chr21:31667299:G>C Detail (hg38) (SOD1)
Information
Genome
| Assembly | Position |
|---|---|
| hg19 | chr21:33,039,612-33,039,612 View the variant detail on this assembly version. |
| hg38 | chr21:31,667,299-31,667,299 |
HGVS
| Type | Transcript | Protein |
|---|---|---|
| RefSeq | NM_000454.4:c.281G>C | NP_000445.1:p.Gly94Ala |
| Ensemble | ENST00000270142.11:c.281G>C | ENST00000270142.11:p.Gly94Ala |
| ENST00000389995.4:c.224G>C | ENST00000389995.4:p.Gly75Ala |
Summary
MGeND
| Clinical significance | |
| Variant entry | |
| GWAS entry | |
| Disease area statistics | Show details |
Disease area statistics
[No Data.]
MGeND
[No Data.]
ClinVar
| Clinical significance | Last evaluated | Review status | Condition | Origin | Links |
|---|---|---|---|---|---|
Pathogenic
|
2022-06-28 | criteria provided, single submitter | amyotrophic lateral sclerosis type 1 |
germline
|
Detail |
CIViC
[No Data.]
DisGeNET
| Score | Disease name | Description | Source | Pubmed | Links |
|---|---|---|---|---|---|
| 0.438 | amyotrophic lateral sclerosis | Blocking gap junction hemichannel significantly suppressed neuronal loss of the ... | BeFree | 21712989 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demet... | BeFree | 12060716 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To learn more about metals in ALS, we determined the metallation states of human... | BeFree | 21068388 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | NA | CLINVAR | Detail | |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demet... | BeFree | 12060716 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demet... | BeFree | 12060716 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants ... | BeFree | 9052802 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We have previously shown that several familial ALS-linked copper-zinc superoxide... | BeFree | 18515363 | Detail |
| 0.001 | Paresis | Transgenic mice carrying familial amyotrophic lateral sclerosis (FALS)-linked mu... | BeFree | 11860498 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In the presence of several of these molecules, A4V and other FALS-linked SOD1 mu... | BeFree | 15738401 | Detail |
| 0.438 | amyotrophic lateral sclerosis | This work characterizes the properties of wild-type (WT) mouse motoneurons in th... | BeFree | 19828728 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Disruption of the structure of the Golgi apparatus and the function of the secre... | BeFree | 15050437 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In this study we have investigated the effects of over-expressing wild-type SOD1... | BeFree | 12531528 | Detail |
| 0.438 | amyotrophic lateral sclerosis | As a model for amyotrophic lateral sclerosis (ALS), transgenic hSOD1(G93A) mice ... | BeFree | 21723884 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The purpose of this study is to assess the effect of rosemary extract and its ma... | BeFree | 19798750 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Human SOD1-G93A specific distribution evidenced in murine brain of a transgenic ... | BeFree | 24579824 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To characterize the cellular response to mitochondrial perturbations at the leve... | BeFree | 21120952 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In the spinal cord of transgenic mice expressing the familial ALS superoxide dis... | BeFree | 18826962 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Blood pressure measurements in a transgenic SOD1-G93A mouse model of amyotrophic... | BeFree | 22424128 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Temporal patterns of cytokine and apoptosis-related gene expression in spinal co... | BeFree | 12124437 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here we show that genetic deficiency of mammalian sterile 20-like kinase 1 (MST1... | BeFree | 23818595 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | This pedigree shows a highly homogenous phenotype which closely resembles the ty... | BeFree | 20176600 | Detail |
| <0.001 | amyotrophic lateral sclerosis | In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant... | BeFree | 25284286 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.001 | amyotrophic lateral sclerosis | Our results demonstrated changes in endocannabinoid signaling, in particular a m... | BeFree | 24703394 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here, we demonstrate that a single dose of tirasemtiv significantly increases su... | BeFree | 24805850 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse mo... | BeFree | 21876739 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here we show that death receptor 6 (DR6) levels are elevated in spinal cords fro... | BeFree | 24113175 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We transplanted human bone marrow mesenchymal stem cells (hMSCs) into the lumbar... | BeFree | 18586098 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Given the protective role of PACAP expression in visceromotor neurons and the op... | BeFree | 23466699 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocyt... | BeFree | 15870071 | Detail |
| <0.001 | Spots on skin | Nine and 36 protein spots displayed elevated and suppressed abundance respective... | BeFree | 15501831 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Furthermore, the extent of sumoylation of EAAT2 does not change during the cours... | BeFree | 24753081 | Detail |
| 0.438 | amyotrophic lateral sclerosis | DJ-1 changes in G93A-SOD1 transgenic mice: implications for oxidative stress in ... | BeFree | 18712292 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Protective effects of resveratrol through the up-regulation of SIRT1 expression ... | BeFree | 21896316 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Galectin-1 accumulation in motor axons occurs before the development of ALS-like... | BeFree | 24707896 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Strains of Saccharomyces cerevisiae that express either the wild type or the amy... | BeFree | 15488469 | Detail |
| 0.002 | acute promyelocytic leukemia | Furthermore, using an astroglial cell line, primary culture of astrocytes, and t... | BeFree | 17823119 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Altogether these findings indicate that G93A-SOD1 expression results in an exace... | BeFree | 22015310 | Detail |
| <0.001 | Exanthema | Nine and 36 protein spots displayed elevated and suppressed abundance respective... | BeFree | 15501831 | Detail |
| 0.438 | amyotrophic lateral sclerosis | These findings suggest that intravenous administration of MSCs significantly imp... | BeFree | 22481270 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The present study was designed to investigate the therapeutic potential of human... | BeFree | 17786603 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Exposure to an environmental neurotoxicant hastens the onset of amyotrophic late... | BeFree | 21586603 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel ... | BeFree | 22987392 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Consistent with this, we have demonstrated that overexpression of Bcl-2, a prote... | BeFree | 10582606 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Since enhanced iron levels are discussed to participate in oxidative stress and ... | BeFree | 23178912 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Pre-symptomatic detection of chronic motor deficits and genotype prediction in c... | BeFree | 19699279 | Detail |
| 0.007 | neuroblastoma | We report that the expression of mutant G93A copper/zinc superoxide dismutase (S... | BeFree | 12901835 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The effect of leukaemia inhibitory factor on SOD1 G93A murine amyotrophic latera... | BeFree | 12967646 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse... | BeFree | 22678056 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Inhibition of glycogen synthase kinase-3 suppresses the onset of symptoms and di... | BeFree | 17433298 | Detail |
| 0.380 | amyotrophic lateral sclerosis | TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model... | BeFree | 25213598 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We have tested the hypothesis that urinary levels of extracellular neurotrophin ... | BeFree | 24475283 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice that overexpress the G93A mutation of the human Cu-Zn superoxide... | BeFree | 18945894 | Detail |
| 0.004 | amyotrophic lateral sclerosis | Because peroxisome proliferator-activated receptor gamma (PPARgamma) agonists ac... | BeFree | 16120782 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Astrocyte dysfunction also occurs in human ALS and transgenic rodents expressing... | BeFree | 18931666 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We suggest that the progressive disruption of M1 corticocortical connections res... | BeFree | 20732897 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We examined the role of MMP-9 in a mouse model of familial ALS and found that la... | BeFree | 17362932 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Adaptive immune neuroprotection in G93A-SOD1 amyotrophic lateral sclerosis mice. | BeFree | 18648532 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Downregulation of the potassium chloride cotransporter KCC2 in vulnerable motone... | BeFree | 20838240 | Detail |
| <0.001 | Neurologic Symptoms | G93A SOD1 transgenic mice show an increase in proteasome activity and induction ... | BeFree | 17482163 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Changes in endocannabinoid receptors and enzymes in the spinal cord of SOD1(G93A... | BeFree | 24703394 | Detail |
| <0.001 | motor neuron disease | Here we determined that dysregulated redox stress in ALS mice caused by NADPH ox... | BeFree | 17853944 | Detail |
| 0.132 | amyotrophic lateral sclerosis | Therefore, we investigate the influence of the soluble factors released by hADSC... | BeFree | 20152807 | Detail |
| 0.081 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Time course of preferential motor unit loss in the SOD1 G93A mouse model of amyo... | BeFree | 17766128 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mou... | BeFree | 18624915 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We established a transgenic mouse line overexpressing the familial ALS-associate... | BeFree | 21345177 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We herein characterised the expression and activity of the glutamate transporter... | BeFree | 19428804 | Detail |
| <0.001 | Mitochondrial cytopathy | Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolat... | BeFree | 18334481 | Detail |
| 0.438 | amyotrophic lateral sclerosis | A major QTL on mouse chromosome 17 resulting in lifespan variability in SOD1-G93... | BeFree | 25008789 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We therefore investigated the effects of lithium and VPA cotreatment on the dise... | BeFree | 18640245 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Immunohistochemical analysis of sympathetic involvement in the SOD1-G93A transge... | BeFree | 23607704 | Detail |
| 0.007 | neuroblastoma | We detected this interaction both in spinal cord extracts of mutant SOD1(G93A) t... | BeFree | 17403032 | Detail |
| 0.123 | muscular atrophy | Transgenic mice overexpressing a mutated form of human SOD1 with a Gly93Ala subs... | BeFree | 11555629 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Amino acids as biomarkers in the SOD1(G93A) mouse model of ALS. | BeFree | 24129262 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To gain new mechanistic insights into the role of these receptors in the context... | BeFree | 22910911 | Detail |
| 0.438 | amyotrophic lateral sclerosis | However, daily administration of dantrolene to mice overexpressing superoxide di... | BeFree | 22750242 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Adenosine A2A receptors activation facilitates neuromuscular transmission in the... | BeFree | 25093813 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To test the hypothesis that inhibitory interneuron innervation of spinal motoneu... | BeFree | 19116365 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Depressed excitability and ion currents linked to slow exocytotic fusion pore in... | BeFree | 25377090 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We show in the SOD1(G93A) mouse model of ALS that recurrent exposure to restrain... | BeFree | 21876068 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Furthermore, we also succeeded in mutant SOD1-specific gene suppression in the l... | BeFree | 20649474 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Based on the temporal correlation of these impairments with the onset of motor w... | BeFree | 9382875 | Detail |
| 0.438 | amyotrophic lateral sclerosis | This finding highlights the utility of the SOD1-G93A transgenic mouse model for ... | BeFree | 9419057 | Detail |
| 0.380 | amyotrophic lateral sclerosis | Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS. | BeFree | 19379791 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Proteomic analysis of the transgenic SOD1(G93A) ALS rat model revealed significa... | BeFree | 16847061 | Detail |
| 0.007 | neuroblastoma | We developed an in vitro disease model with motor neuron-neuroblastoma hybrid ce... | BeFree | 15289674 | Detail |
| 0.081 | amyotrophic lateral sclerosis | Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel ... | BeFree | 22987392 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mou... | BeFree | 18624915 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Neuroprotective effects of diallyl trisulfide in SOD1-G93A transgenic mouse mode... | BeFree | 21147075 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF... | BeFree | 22390177 | Detail |
| 0.438 | amyotrophic lateral sclerosis | However, no systematic studies focusing on such effects using ALS models other t... | BeFree | 22438926 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In an ALS mouse model carrying the human Cu/Zn superoxide dismutase (hSOD1) G93A... | BeFree | 18054961 | Detail |
| <0.001 | cholera | Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show tha... | BeFree | 24708899 | Detail |
| 0.164 | motor neuron disease | In the present study, we investigated the time course of microglial (major histo... | BeFree | 9633809 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Astrocytes expressing the ALS-linked SOD1(G93A) mutation display a decreased mit... | BeFree | 22509356 | Detail |
| 0.007 | Central neuroblastoma | In a recent work, we have observed that calcineurin activity is depressed in two... | BeFree | 11701756 | Detail |
| 0.007 | neuroblastoma | In a recent work, we have observed that calcineurin activity is depressed in two... | BeFree | 11701756 | Detail |
| 0.438 | amyotrophic lateral sclerosis | While there are legitimate concerns about the physiological differences between ... | BeFree | 22117132 | Detail |
| 0.438 | amyotrophic lateral sclerosis | TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model... | BeFree | 25213598 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this work, we aimed to investigate whether transplanted MN could prevent moto... | BeFree | 19660174 | Detail |
| 0.007 | Central neuroblastoma | We detected this interaction both in spinal cord extracts of mutant SOD1(G93A) t... | BeFree | 17403032 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Limited effects of glatiramer acetate in the high-copy number hSOD1-G93A mouse m... | BeFree | 17597611 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A... | BeFree | 24911596 | Detail |
| 0.164 | motor neuron disease | We therefore studied whether motor neuron disease (MND) in mice expressing the h... | BeFree | 11011020 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The neuroprotective potential of those two genes was further investigated in viv... | BeFree | 23732987 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Therefore, in the present study, we investigated the process of motor neuron deg... | BeFree | 20553309 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In the present study, we used the superoxide dismutase 1(SODI)(G93A) transgenic ... | BeFree | 12770687 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study we analyzed the effects of ALS on bone using the well established ... | BeFree | 25648889 | Detail |
| <0.001 | cholera | Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show tha... | BeFree | 24708899 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To investigate whether GOS could provide neuroprotective effect in ALS, we appli... | BeFree | 23673277 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Experiments were performed by inducing exocytosis in wild-type (WT) mice, in amp... | BeFree | 25497732 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant... | BeFree | 25284286 | Detail |
| 0.438 | amyotrophic lateral sclerosis | This study attempted to determine the effect of HUCB on SOD1 mice (transgenic B6... | BeFree | 10896029 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We show here that the expression of caspase-11 is upregulated in the spinal cord... | BeFree | 12843244 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We employed ALS model SOD1(G93A) mice and GlcNAc6ST-1(-/-) mice, which are KS-de... | BeFree | 23825599 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Moreover, we demonstrate that upon expression in yeast cells the common ALS-link... | BeFree | 21055392 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting ... | BeFree | 23405225 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The lack of effect of specific overexpression of IGF-1 in the central nervous sy... | BeFree | 17597610 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Ventral roots from SOD1(G93A) ALS mice were analyzed by label-free quantitative ... | BeFree | 20698585 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In a recent work, we have observed that calcineurin activity is depressed in two... | BeFree | 11701756 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Dose-dependent efficacy of ALS-human mesenchymal stem cells transplantation into... | BeFree | 19879334 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.438 | amyotrophic lateral sclerosis | These results indicate that local complement activation and increased expression... | BeFree | 24067070 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Characterization of human sporadic ALS biomarkers in the familial ALS transgenic... | BeFree | 23836781 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here, we examine the potential contribution of peripheral axon ensheathing Schwa... | BeFree | 20008901 | Detail |
| 0.004 | amyotrophic lateral sclerosis | Proteomic analysis of the transgenic SOD1(G93A) ALS rat model revealed significa... | BeFree | 16847061 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocyt... | BeFree | 15870071 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS. | BeFree | 19379791 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Taken together, this suggests that reduced levels of VGLUT2 decrease motor neuro... | BeFree | 19770042 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The pathogenic SOD1 variants A4V and G93A have been the foci of multiple studies... | BeFree | 19800308 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Zinc pre-treatment enhances NMDAR-mediated excitotoxicity in cultured cortical n... | BeFree | 21056589 | Detail |
| 0.438 | amyotrophic lateral sclerosis | A structure activity relationship of a lead scaffold exhibiting neuroprotective ... | BeFree | 23021992 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this article, we report a proteomic analysis using a widely used ALS mouse mo... | BeFree | 19438725 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice that express a mutant Cu,Zn-SOD, Gly93 --> Ala (G93A), have b... | BeFree | 10102580 | Detail |
| 0.007 | neuroblastoma | We demonstrated that expression of the fully active G93A Cu,Zn superoxide dismut... | BeFree | 12753090 | Detail |
| 0.002 | amyotrophic lateral sclerosis | The lack of effect of specific overexpression of IGF-1 in the central nervous sy... | BeFree | 17597610 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Therefore, we investigate the influence of the soluble factors released by hADSC... | BeFree | 20152807 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We studied the effect of a high (HEX) and moderate-level exercise (MEX) on body ... | BeFree | 19968977 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We conclude that miR-206 is elevated in the circulation of symptomatic SOD1-G93A... | BeFree | 24586506 | Detail |
| 0.007 | Central neuroblastoma | Impairment of nuclear factor-kappaB activation increased glutamate excitotoxicit... | BeFree | 20623531 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We have compared FMO mRNA expression in the control mouse strain C57BL/6J and in... | BeFree | 21082301 | Detail |
| 0.438 | amyotrophic lateral sclerosis | A transgenic mouse model of amyotrophic lateral sclerosis was selected based on ... | BeFree | 23383983 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To elucidate the underlying molecular events, we used rapid charge-cooled device... | BeFree | 19060114 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We examined protein levels of the glutamate transporters GLT-1, GLAST and EAAC1 ... | BeFree | 11790392 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice carrying the G93A mutation of the superoxide dismutase 1 (SOD1) ... | BeFree | 20450936 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Degeneration of corticospinal and bulbospinal systems in the superoxide dismutas... | BeFree | 12384220 | Detail |
| 0.164 | motor neuron disease | To test whether ALS2 plays a protective role against mutant SOD1-mediated motor ... | BeFree | 16973244 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice overexpressing the human mutated form (G93A) of Cu,Zn-superoxide... | BeFree | 14625013 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In the current study, we have examined the therapeutic efficacy of memantine in ... | BeFree | 16262676 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| <0.001 | Immunologic Deficiency Syndromes | Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A... | BeFree | 24911596 | Detail |
| 0.007 | Central neuroblastoma | We developed motor neuron-neuroblastoma hybrid cells that expressed a mutant (G9... | BeFree | 12392756 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Thus, hSOD1-G93A mutation causes both motor and sensory neuropathies, and as suc... | BeFree | 19293633 | Detail |
| 0.007 | Central neuroblastoma | To characterize the cellular response to mitochondrial perturbations at the leve... | BeFree | 21120952 | Detail |
| 0.003 | leukemia | The effect of leukaemia inhibitory factor on SOD1 G93A murine amyotrophic latera... | BeFree | 12967646 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Our results indicate that the current density at high GABA concentrations is sim... | BeFree | 18973555 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-... | BeFree | 8610185 | Detail |
| <0.001 | motor neuron disease | In the present study, we investigated the time course of microglial (major histo... | BeFree | 9633809 | Detail |
| 0.438 | amyotrophic lateral sclerosis | This study was undertaken to evaluate the effect of EGCG on ALS model mice with ... | BeFree | 16356650 | Detail |
| 0.121 | amyotrophic lateral sclerosis | The present study provides the first evidence that MnSOD immunoreactivity was in... | BeFree | 14568347 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In the present study, we injected MSCs into the cerebrospinal fluid of symptomat... | BeFree | 19267424 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The relevance of identified genes as clinical biomarkers was tested in the perip... | BeFree | 20530642 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse ... | BeFree | 24023695 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study, we demonstrate complement system involvement in a rodent model of... | BeFree | 19050293 | Detail |
| <0.001 | cholera | Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show tha... | BeFree | 24708899 | Detail |
| 0.007 | neuroblastoma | In order to investigate the effect of various calcium modulators and the SOD-1 m... | BeFree | 12151755 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We studied the behaviors of spinal neurons using an in vitro preparation of the ... | BeFree | 19955354 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We examined protein levels of the glutamate transporters GLT-1, GLAST and EAAC1 ... | BeFree | 11790392 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To test a new therapeutic strategy for ALS, we examined the effect of caspase in... | BeFree | 10764647 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In the present study, we demonstrate an age-dependent increase in Bcl-xL and Ets... | BeFree | 19179380 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Galectin-1 accumulation in motor axons occurs before the development of ALS-like... | BeFree | 24707896 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Increased number and differentiation of neural precursor cells in the brainstem ... | BeFree | 17439705 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Leukemia inhibitory factor by systemic administration rescues spinal motor neuro... | BeFree | 11730713 | Detail |
| 0.081 | amyotrophic lateral sclerosis | Progressive loss of a glial potassium channel (KCNJ10) in the spinal cord of the... | BeFree | 16925593 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mo... | BeFree | 21620832 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Phenotype of transgenic mice carrying a very low copy number of the mutant human... | BeFree | 24945277 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study, we analyzed the spatiotemporal alterations of phospholipid compos... | BeFree | 25841322 | Detail |
| 0.007 | motor neuron disease | To test whether ALS2 plays a protective role against mutant SOD1-mediated motor ... | BeFree | 16973244 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To better understand the autonomic involvement in ALS we measured autonomic func... | BeFree | 20869331 | Detail |
| 0.003 | amyotrophic lateral sclerosis | Erythropoietin modulates the immune-inflammatory response of a SOD1(G93A) transg... | BeFree | 24820540 | Detail |
| 0.022 | Neurodegenerative Disorders | Transgenic mice carrying the G93A mutation of the superoxide dismutase 1 (SOD1) ... | BeFree | 20450936 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Functional over-load saves motor units in the SOD1-G93A transgenic mouse model o... | BeFree | 19879358 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Metformin treatment has no beneficial effect in a dose-response survival study i... | BeFree | 21909419 | Detail |
| 0.438 | amyotrophic lateral sclerosis | D-serine is increased two-fold in spinal cords of G93A Cu,Zn-superoxide dismutas... | BeFree | 22117694 | Detail |
| <0.001 | Spinal Cord Ischemia | Even though the single transgenic hHsp27 mice showed protection against spinal c... | BeFree | 18624915 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Gonadectomy and dehydroepiandrosterone (DHEA) do not modulate disease progressio... | BeFree | 22409357 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Changes in Wnt5a and Fzd2 expression in the spinal cord of SOD1(G93A) transgenic... | BeFree | 23826406 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We determined the expression of Wnt3a, β-catenin, and Cyclin D1 in the adult spi... | BeFree | 22426476 | Detail |
| 0.011 | Immunologic Deficiency Syndromes | Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A... | BeFree | 24911596 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Using 1H-15N HSQC NMR spectroscopy, we have analyzed hydrogen exchange at the am... | BeFree | 19655787 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We then investigated whether SA4503 suppressed the progression of ALS in an SOD1... | BeFree | 24334165 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Behavioural and anatomical effects of systemically administered leukemia inhibit... | BeFree | 12915243 | Detail |
| <0.001 | Cytochrome-c Oxidase Deficiency | In contrast, CCS/G93A SOD1 mouse spinal cord showed no reduction in levels of se... | BeFree | 18334481 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The present study thus provides direct evidence of activated UPR in motor neuron... | BeFree | 22390177 | Detail |
| 0.007 | neuroblastoma | To understand better the role of these mutations in the pathophysiology of FALS ... | BeFree | 17979159 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Although up-regulation of caspase-12 has been reported in G93A SOD1 transgenic m... | BeFree | 20816908 | Detail |
| 0.164 | motor neuron disease | Since the mechanism whereby mutations in SOD1 lead to MND remains enigmatic, we ... | BeFree | 9382875 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We here investigated the level of activation of the Ca(2+)-dependent protease ca... | BeFree | 25151305 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | We report that the expression of mutant G93A copper/zinc superoxide dismutase (S... | BeFree | 12901835 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Acute glial activation by stab injuries does not lead to overt damage or motor n... | BeFree | 20005223 | Detail |
| 0.006 | amyotrophic lateral sclerosis | Upregulation of amyloid beta (A4) precursor protein (APP) in muscle fibres coinc... | BeFree | 22678056 | Detail |
| 0.080 | amyotrophic lateral sclerosis | These results indicate that local complement activation and increased expression... | BeFree | 24067070 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Background and gender effects on survival in the TgN(SOD1-G93A)1Gur mouse model ... | BeFree | 16024047 | Detail |
| <0.001 | Gastric hemorrhage | Gastric bleeding was not induced by AAD-2004 even at a dose 400-fold higher than... | BeFree | 22537108 | Detail |
| 0.022 | Neurodegenerative Disorders | We used SOD1(G93A) mice as a model, which exhibit the motor neuron-specific neur... | BeFree | 23470532 | Detail |
| 0.002 | amyotrophic lateral sclerosis | Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF... | BeFree | 22390177 | Detail |
| 0.438 | amyotrophic lateral sclerosis | On the relation of oxidative stress to neuroinflammation: lessons learned from t... | BeFree | 17034351 | Detail |
| 0.007 | Central neuroblastoma | To understand better the role of these mutations in the pathophysiology of FALS ... | BeFree | 17979159 | Detail |
| 0.164 | motor neuron disease | Here we determined that dysregulated redox stress in ALS mice caused by NADPH ox... | BeFree | 17853944 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Treatments to rescue motor neurons according to dying-forward model of motor neu... | BeFree | 21057983 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Long-term (10-11 weeks) transplantation of hNT Neurons into the L(4)-L(5) segmen... | BeFree | 11922659 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice overexpressing a mutant human SOD1 gene (G93A-SOD1) were used to... | BeFree | 15634778 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse mo... | BeFree | 15519671 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mou... | BeFree | 18624915 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Neuroprotective effects of JGK-263 in transgenic SOD1-G93A mice of amyotrophic l... | BeFree | 24680562 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this work we evaluated the locomotor performance of the SOD1(G93A) mouse mode... | BeFree | 21733494 | Detail |
| 0.007 | neuroblastoma | Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A)... | BeFree | 22875931 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Arylsulfanyl pyrazolones block mutant SOD1-G93A aggregation. Potential applicati... | BeFree | 21095130 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Using an ALS mouse model carrying a high copy number of a mutant human superoxid... | BeFree | 22472631 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The finding of concomitant dysfunction of upper and lower motoneurons contribute... | BeFree | 21354365 | Detail |
| <0.001 | Compression of spinal cord | The human G93A-SOD1 mutation in a pre-symptomatic rat model of amyotrophic later... | BeFree | 21078175 | Detail |
| <0.001 | motor neuron disease | Here we determined that dysregulated redox stress in ALS mice caused by NADPH ox... | BeFree | 17853944 | Detail |
| 0.002 | amyotrophic lateral sclerosis | Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocyt... | BeFree | 15870071 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We employed ALS model SOD1(G93A) mice and GlcNAc6ST-1(-/-) mice, which are KS-de... | BeFree | 23825599 | Detail |
| 0.438 | amyotrophic lateral sclerosis | ) delivery of recombinant vascular endothelial growth factor (Vegf) in a SOD1(G9... | BeFree | 15568021 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | G93A Cu/Zn superoxide dismutase (SOD1), a human mutant SOD1 associated with fami... | BeFree | 16624679 | Detail |
| <0.001 | Pituitary Diseases | Specifically, we observe aberrant endogenous pulsatile GH secretion, reduced pit... | BeFree | 22621959 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The purpose of this study was to determine structural changes which trigger the ... | BeFree | 17039412 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn supero... | BeFree | 18485920 | Detail |
| 0.002 | Presenile dementia | We suggest that the progressive disruption of M1 corticocortical connections res... | BeFree | 20732897 | Detail |
| 0.001 | Mitochondrial pathology | Our study provides a new mechanistic link for hSOD1(G93A)-mediated impairment of... | BeFree | 26182418 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We established a transgenic mouse line overexpressing the familial ALS-associate... | BeFree | 21345177 | Detail |
| 0.007 | Central neuroblastoma | We developed an in vitro disease model with motor neuron-neuroblastoma hybrid ce... | BeFree | 15289674 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The present study provides the first in vivo evidence that alpha-synuclein immun... | BeFree | 12757887 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In vivo quantification of spinal and bulbar motor neuron degeneration in the G93... | BeFree | 16740261 | Detail |
| <0.001 | Dissecting aneurysm of the thoracic aorta | Analysis of motor-evoked potentials recorded from the thoracic spinal cord of sy... | BeFree | 22916141 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here, we demonstrate that the interferon (IFN) signaling pathway is activated in... | BeFree | 21446050 | Detail |
| 0.002 | AMYOTROPHIC LATERAL SCLEROSIS 1 | We reported that Cu,Zn-SOD can catalyze free radical generation and a FALS mutan... | BeFree | 9083002 | Detail |
| 0.022 | Neurodegenerative Disorders | We then analyzed molecular changes in microglia during neurodegenerative disease... | BeFree | 23850290 | Detail |
| 0.438 | amyotrophic lateral sclerosis | PACAP signaling exerts opposing effects on neuroprotection and neuroinflammation... | BeFree | 23466699 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To test allele-specific silencing in vivo, we made transgenic mice that express ... | BeFree | 16857362 | Detail |
| 0.007 | neuroblastoma | We developed motor neuron-neuroblastoma hybrid cells that expressed a mutant (G9... | BeFree | 12392756 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Our data indicate that the clinically relevant SOD1 G93A mutation, which leads t... | BeFree | 15350647 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Excitability properties of mouse motor axons in the mutant SOD1(G93A) model of a... | BeFree | 20095022 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Presymptomatic treatment with acetylcholinesterase antisense oligonucleotides pr... | BeFree | 24455732 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Therefore, NSC-34/hSOD1(G93A) cells are a suitable cell model to study GA dysfun... | BeFree | 18004513 | Detail |
| 0.007 | neuroblastoma | To characterize the cellular response to mitochondrial perturbations at the leve... | BeFree | 21120952 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The transgenic SOD1(G93A) mouse is a model of human amyotrophic lateral sclerosi... | BeFree | 24239688 | Detail |
| 0.001 | Mitochondrial pathology | Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological defici... | BeFree | 17389365 | Detail |
| 0.007 | Central neuroblastoma | We demonstrated that expression of the fully active G93A Cu,Zn superoxide dismut... | BeFree | 12753090 | Detail |
| <0.001 | Mitochondrial Myopathies | Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolat... | BeFree | 18334481 | Detail |
| 0.164 | motor neuron disease | These findings suggest that the expression of the SOD1(G93A G1H) mutant protein ... | BeFree | 12384220 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Since glutamate toxicity has been proposed as a mechanism for the degeneration o... | BeFree | 20060132 | Detail |
| <0.001 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To assess molecular pathological effects of the anti-inflammatory peroxisome pro... | BeFree | 18312546 | Detail |
| <0.001 | Mitochondrial pathology | G93A SOD1 transgenic mice overexpressing CCS protein develop an accelerated dise... | BeFree | 18334481 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study, we demonstrated that the level of 5-MTHF is significantly decreas... | BeFree | 20334883 | Detail |
| <0.001 | amyotrophic lateral sclerosis | These findings provide the first evidence of SP having a physiological and prote... | BeFree | 21726643 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic rats expressing a mutated form of the human Cu/Zn superoxide dismutas... | BeFree | 16540243 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Leukemia inhibitory factor by systemic administration rescues spinal motor neuro... | BeFree | 11730713 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop... | BeFree | 16049935 | Detail |
| 0.001 | Terminal illness | Altered expression of astroglial glutamate transporters accompanied by reduced c... | BeFree | 12629173 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Cross-breeding of SOD1(G93A) ALS mice with asic1a-deficient mice delayed the ons... | BeFree | 23306556 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Low levels of SMN also occur in models of amyotrophic lateral sclerosis (ALS) ca... | BeFree | 24210254 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Relationship between neuropathology and disease progression in the SOD1(G93A) AL... | BeFree | 21145892 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In transgenic mice carrying the G93A human mutation of Cu/Zn superoxide dismutas... | BeFree | 12421599 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Experiments were performed by inducing exocytosis in wild-type (WT) mice, in amp... | BeFree | 25497732 | Detail |
| 0.164 | motor neuron disease | Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop... | BeFree | 16049935 | Detail |
| 0.001 | Motor symptoms | Early changes of neuromuscular transmission in the SOD1(G93A) mice model of ALS ... | BeFree | 24040091 | Detail |
| 0.438 | amyotrophic lateral sclerosis | G93A SOD1 alters cell cycle in a cellular model of Amyotrophic Lateral Sclerosis... | BeFree | 20561900 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Increased ceramide, glucosylceramide, GM3, and hexosaminidase activity were also... | BeFree | 26056266 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The present study provides the first evidence that MnSOD immunoreactivity was in... | BeFree | 14568347 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We report that symptomatic male hSOD1(G93A) transgenic mice exhibit a deficiency... | BeFree | 22621959 | Detail |
| 0.081 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The SOD1 transgene in the G93A mouse model of amyotrophic lateral sclerosis lies... | BeFree | 16036435 | Detail |
| 0.002 | Promyelocytic leukemia | Furthermore, using an astroglial cell line, primary culture of astrocytes, and t... | BeFree | 17823119 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mou... | BeFree | 18624915 | Detail |
| 0.001 | amyotrophic lateral sclerosis | Protective effects of resveratrol through the up-regulation of SIRT1 expression ... | BeFree | 21896316 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.005 | dementia | We suggest that the progressive disruption of M1 corticocortical connections res... | BeFree | 20732897 | Detail |
| 0.164 | motor neuron disease | Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-... | BeFree | 8610185 | Detail |
| <0.001 | Cytochrome-c Oxidase Deficiency | Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS pr... | BeFree | 18334481 | Detail |
| 0.007 | Central neuroblastoma | Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A)... | BeFree | 22875931 | Detail |
| 0.002 | Impaired cognition | Here, we examine whether prefrontal cortex neuronal abnormalities and related co... | BeFree | 18081837 | Detail |
| 0.438 | amyotrophic lateral sclerosis | G93A SOD1 expression in motor neurons of C. elegans results in characteristic al... | BeFree | 23503474 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Protein interactions of the Amyotrophic Lateral Sclerosis (ALS)-linked copper-zi... | BeFree | 18652897 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Lentivirus and adeno-associated virus have been used to knockdown levels of muta... | BeFree | 19495690 | Detail |
| 0.438 | amyotrophic lateral sclerosis | delivery of Ara-C accelerated disease progression in SOD1(G93A) mouse model of A... | BeFree | 22523565 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We recently established that the SOD1-G93A transgenic mouse is a suitable model ... | BeFree | 19495873 | Detail |
| 0.438 | amyotrophic lateral sclerosis | By placing running wheels in each mouse's cage to measure their exercise activit... | BeFree | 19418268 | Detail |
| 0.121 | Muscle Weakness | We show in the SOD1(G93A) mouse model of ALS that recurrent exposure to restrain... | BeFree | 21876068 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Genetic biomarkers for ALS disease in transgenic SOD1(G93A) mice. | BeFree | 22412900 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To comparatively evaluate the therapeutic potentials of human bone marrow-derive... | BeFree | 17510731 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We therefore compared onset and progression of disease symptoms in female and ma... | BeFree | 18702691 | Detail |
| 0.122 | amyotrophic lateral sclerosis | In the present study, we demonstrate an age-dependent increase in Bcl-xL and Ets... | BeFree | 19179380 | Detail |
| <0.001 | amyotrophic lateral sclerosis | In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant... | BeFree | 25284286 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Recently we studied glycine receptors (GlyRs) in motoneurons in an ALS mouse mod... | BeFree | 21558795 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal co... | BeFree | 16616851 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Altered expression of DJ-1 and PINK1 in sporadic ALS and in the SOD1(G93A) ALS m... | BeFree | 24128678 | Detail |
| <0.001 | Helminthiasis | Crossing the daf-2(e1370) mutation into the G93A SOD1 mutant worms significantly... | BeFree | 23503474 | Detail |
| 0.008 | Pituitary Diseases | Specifically, we observe aberrant endogenous pulsatile GH secretion, reduced pit... | BeFree | 22621959 | Detail |
| 0.001 | Terminal illness | Female SOD1(G93A) mice housed with environmental enrichment were found to reach ... | BeFree | 18702691 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here, we tested the potential neuroprotective effects of UCP2 and its ability to... | BeFree | 24141050 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 m... | BeFree | 17432958 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Recombinant human erythropoietin suppresses symptom onset and progression of G93... | BeFree | 17439481 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | In parallel, MSCs derived from the bone marrow of a transgenic rat model of fami... | BeFree | 18539273 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Immunohistochemical study on the distribution of insulin-like growth factor-bind... | BeFree | 18823765 | Detail |
| <0.001 | Muscle degeneration | Results indicate that while whisking still occurs in SOD1(G93A) mice and is rela... | BeFree | 24239688 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The basal glutamate efflux from spinal cord nerve terminals of mice-expressing h... | BeFree | 21175617 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Functional neural stem cell isolation from brains of adult mutant SOD1 (SOD1(G93... | BeFree | 20810028 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Abnormal medial prefrontal cortex connectivity and defective fear extinction in ... | BeFree | 18081837 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Identification of compounds protective against G93A-SOD1 toxicity for the treatm... | BeFree | 21073276 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We report here that a familial mouse model (transgenic mice over-expressing the ... | BeFree | 23754387 | Detail |
| 0.001 | Terminal illness | Fast-twitch and slow-twitch muscles from symptomatic SOD1-G93A mice (age 90 days... | BeFree | 24586506 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The work is a continuation of studies on tau expression and alternative splicing... | BeFree | 17268853 | Detail |
| <0.001 | amyotrophic lateral sclerosis | The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine f... | BeFree | 12684256 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The precise mechanism by which this receptor drives the apoptotic process, both ... | BeFree | 11771768 | Detail |
| 0.438 | amyotrophic lateral sclerosis | As proof of concept, we use the IS paradigm to express murine Interleukin (IL)-1... | BeFree | 25228069 | Detail |
| <0.001 | amyotrophic lateral sclerosis | Taken together, this suggests that reduced levels of VGLUT2 decrease motor neuro... | BeFree | 19770042 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Recently, we reported that a subpopulation of spinal cord astrocytes degenerates... | BeFree | 22072391 | Detail |
| 0.126 | brain ischemia | The superoxide dismutase1 (SOD1) G93A mutation does not promote neuronal injury ... | BeFree | 15350647 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Loss of a single functional Sil1 allele in an ALS mouse model (SOD1-G93A) enhanc... | BeFree | 25559081 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The human G93A-SOD1 mutation in a pre-symptomatic rat model of amyotrophic later... | BeFree | 21078175 | Detail |
| 0.438 | amyotrophic lateral sclerosis | By combining immuno-spin trapping and molecular magnetic resonance imaging, in v... | BeFree | 23722162 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Autophagy activation and neuroprotection by progesterone in the G93A-SOD1 transg... | BeFree | 23891729 | Detail |
| 0.438 | amyotrophic lateral sclerosis | These findings provide the first evidence of SP having a physiological and prote... | BeFree | 21726643 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate e... | BeFree | 15885796 | Detail |
| 0.002 | amyotrophic lateral sclerosis | Here we investigated the expression of NF-κB and the main UPR markers X-box bind... | BeFree | 24666819 | Detail |
| 0.002 | amyotrophic lateral sclerosis | Although viral delivery of IGF-I has shown therapeutic efficacy in the SOD1(G93A... | BeFree | 19038252 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Point mutations such as G93A and A4V in the human Cu/Zn-superoxide dismutase gen... | BeFree | 16045483 | Detail |
| 0.164 | motor neuron disease | We next applied CME repeatedly to living Wld(S) mice and to SOD1(G93A) mice, an ... | BeFree | 19683573 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Our previous study showed that glycinergic innervation of spinal motoneurons is ... | BeFree | 21414903 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Three ALS mice [(SOD1-G93A)1Gur] and three controls [(SOD-1)2Gur] were obtained ... | BeFree | 9527889 | Detail |
| 0.164 | motor neuron disease | To determine where and when the pathological changes of motor neuron disease beg... | BeFree | 14736504 | Detail |
| 0.003 | glioblastoma | Furthermore, selective death of embryonal spinal motor neurons from G93A-SOD1 tr... | BeFree | 15208263 | Detail |
| 0.001 | Mitochondrial pathology | G93A SOD1 transgenic mice overexpressing CCS protein develop an accelerated dise... | BeFree | 18334481 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We demonstrate that transient genetic manipulation of zebrafish to express G93A-... | BeFree | 22938571 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Effect of genetic background on onset and disease progression in the SOD1-G93A m... | BeFree | 22424126 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To evaluate the therapeutic potential of human neural progenitor cells (hNPs) in... | BeFree | 19322031 | Detail |
| <0.001 | Kearns-Sayre syndrome | Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolat... | BeFree | 18334481 | Detail |
| 0.001 | Motor symptoms | Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor sympto... | BeFree | 16478527 | Detail |
| <0.001 | AMYOTROPHIC LATERAL SCLEROSIS 1 | To clarify the biological significance of the interaction of the redox system (P... | BeFree | 14648077 | Detail |
| 0.438 | amyotrophic lateral sclerosis | The dietary exposure to EPA (300 mg/kg/day) in a well-established mouse model of... | BeFree | 23620776 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | To clarify the biological significance of the interaction of the redox system (P... | BeFree | 14648077 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | The paper by Butterfield and colleagues reporting the use of redox proteomics to... | BeFree | 17603925 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Disease progression of human SOD1 (G93A) transgenic ALS model rats. | BeFree | 16342121 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Measuring early pre-symptomatic changes in locomotion of SOD1-G93A rats--a roden... | BeFree | 18824029 | Detail |
| 0.022 | Neurodegenerative Disorders | These results show that in the SOD1(G93A) model of neurodegenerative diseases, t... | BeFree | 20060132 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study we have analysed the effect of Monocyte Chemoattractant Protein-1 ... | BeFree | 23752092 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Our results demonstrated that caprylic triglyceride alleviates ALS-type motor im... | BeFree | 23145119 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Innate and adaptive immunity were investigated in the CNS of the Superoxide Dism... | BeFree | 18997009 | Detail |
| <0.001 | Deglutition Disorders | We recently established that the SOD1-G93A transgenic mouse is a suitable model ... | BeFree | 19495873 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A... | BeFree | 24911596 | Detail |
| 0.132 | amyotrophic lateral sclerosis | Furthermore, the extent of sumoylation of EAAT2 does not change during the cours... | BeFree | 24753081 | Detail |
| 0.007 | Central neuroblastoma | We report that the expression of mutant G93A copper/zinc superoxide dismutase (S... | BeFree | 12901835 | Detail |
| 0.520 | AMYOTROPHIC LATERAL SCLEROSIS 1 | Behavioural and anatomical effects of systemically administered leukemia inhibit... | BeFree | 12915243 | Detail |
| 0.438 | amyotrophic lateral sclerosis | To gain insights on the significance of BIs we asked whether neurons with BIs ha... | BeFree | 11907804 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Erythropoietin modulates the immune-inflammatory response of a SOD1(G93A) transg... | BeFree | 24820540 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Vacuolization correlates with spin-spin relaxation time in motor brainstem nucle... | BeFree | 17868365 | Detail |
| 0.001 | Motor symptoms | In addition, we have recently reported elevated heart rate and blood pressure in... | BeFree | 23607704 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In mice that express the mutant human gene Cu/Zn superoxide dismutase G93A (SOD1... | BeFree | 12668759 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In this study, we examined the regulation of glutamate transporters by type 5 me... | BeFree | 16371010 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Glycoprotein nonmetastatic melanoma protein B (GPNMB) was identified as an ALS-r... | BeFree | 22891158 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Investigation of the G93A-SOD1 mouse model for ALS revealed increased message an... | BeFree | 15379894 | Detail |
| 0.127 | ischemia | Early vulnerability to ischemia/reperfusion injury in motor terminals innervatin... | BeFree | 17292357 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Mice over-expressing the mutant human G93A-SOD1 are widely used as an animal mod... | BeFree | 15371748 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Because peroxisome proliferator-activated receptor gamma (PPARgamma) agonists ac... | BeFree | 16120782 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Mice bearing the mutated gene for Cu/Zn superoxide dismutase (G93A) are a good m... | BeFree | 19296491 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Transgenic mice expressing a mutated (G93A) human Cu/Zn superoxide dismutase (SO... | BeFree | 10737625 | Detail |
| 0.001 | Motor symptoms | After identification of the clinical onset in each female G93A mutant SOD1 trans... | BeFree | 18718468 | Detail |
| 0.007 | Central neuroblastoma | In order to investigate the effect of various calcium modulators and the SOD-1 m... | BeFree | 12151755 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Here we report that a single injection of a VEGF-expressing lentiviral vector in... | BeFree | 15164063 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Progressive loss of a glial potassium channel (KCNJ10) in the spinal cord of the... | BeFree | 16925593 | Detail |
| 0.007 | neuroblastoma | Impairment of nuclear factor-kappaB activation increased glutamate excitotoxicit... | BeFree | 20623531 | Detail |
| 0.001 | amyotrophic lateral sclerosis | Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF... | BeFree | 22390177 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In superoxide dismutase 1(G93A) transgenic mouse model of ALS, AAD-2004 blocked ... | BeFree | 22537108 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Although viral delivery of IGF-I has shown therapeutic efficacy in the SOD1(G93A... | BeFree | 19038252 | Detail |
| 0.438 | amyotrophic lateral sclerosis | Mice engineered to express a transgene encoding a human Cu/Zn superoxide dismuta... | BeFree | 9382875 | Detail |
| 0.438 | amyotrophic lateral sclerosis | In the superoxide dismutase 1 (SOD1)-G93A mouse model of amyotrophic lateral scl... | BeFree | 21346327 | Detail |
| 0.438 | amyotrophic lateral sclerosis | We characterized synaptosomal glutamate transport activity in a recently develop... | BeFree | 12629173 | Detail |
Annotation
Annotations
| Descrption | Source | Links |
|---|---|---|
| NM_000454.5(SOD1):c.281G>C (p.Gly94Ala) AND Amyotrophic lateral sclerosis type 1 | ClinVar | Detail |
| Blocking gap junction hemichannel significantly suppressed neuronal loss of the spinal cord and exte... | DisGeNET | Detail |
| Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demetallation and that he... | DisGeNET | Detail |
| To learn more about metals in ALS, we determined the metallation states of human wild-type or mutant... | DisGeNET | Detail |
| NA | DisGeNET | Detail |
| Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demetallation and that he... | DisGeNET | Detail |
| Herein, we demonstrate that the entry of SOD1 into mitochondria depends on demetallation and that he... | DisGeNET | Detail |
| High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and G37R were p... | DisGeNET | Detail |
| We have previously shown that several familial ALS-linked copper-zinc superoxide dismutase (SOD1) mu... | DisGeNET | Detail |
| Transgenic mice carrying familial amyotrophic lateral sclerosis (FALS)-linked mutant Cu/Zn superoxid... | DisGeNET | Detail |
| In the presence of several of these molecules, A4V and other FALS-linked SOD1 mutants such as G93A a... | DisGeNET | Detail |
| This work characterizes the properties of wild-type (WT) mouse motoneurons in the second postnatal w... | DisGeNET | Detail |
| Disruption of the structure of the Golgi apparatus and the function of the secretory pathway by muta... | DisGeNET | Detail |
| In this study we have investigated the effects of over-expressing wild-type SOD1 and two mutant form... | DisGeNET | Detail |
| As a model for amyotrophic lateral sclerosis (ALS), transgenic hSOD1(G93A) mice constitute the stand... | DisGeNET | Detail |
| The purpose of this study is to assess the effect of rosemary extract and its major constituents, ro... | DisGeNET | Detail |
| Human SOD1-G93A specific distribution evidenced in murine brain of a transgenic model for amyotrophi... | DisGeNET | Detail |
| To characterize the cellular response to mitochondrial perturbations at the level of gene expression... | DisGeNET | Detail |
| In the spinal cord of transgenic mice expressing the familial ALS superoxide dismutase 1 (SOD1) gene... | DisGeNET | Detail |
| Blood pressure measurements in a transgenic SOD1-G93A mouse model of amyotrophic lateral sclerosis. | DisGeNET | Detail |
| Temporal patterns of cytokine and apoptosis-related gene expression in spinal cords of the G93A-SOD1... | DisGeNET | Detail |
| Here we show that genetic deficiency of mammalian sterile 20-like kinase 1 (MST1) delays disease ons... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| This pedigree shows a highly homogenous phenotype which closely resembles the typical phenotype of s... | DisGeNET | Detail |
| In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant transgenic mice bef... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| Our results demonstrated changes in endocannabinoid signaling, in particular a marked up-regulation ... | DisGeNET | Detail |
| Here, we demonstrate that a single dose of tirasemtiv significantly increases submaximal isometric f... | DisGeNET | Detail |
| Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic l... | DisGeNET | Detail |
| Here we show that death receptor 6 (DR6) levels are elevated in spinal cords from post-mortem sample... | DisGeNET | Detail |
| We transplanted human bone marrow mesenchymal stem cells (hMSCs) into the lumbar spinal cord of asym... | DisGeNET | Detail |
| Given the protective role of PACAP expression in visceromotor neurons and the opposing effect on mic... | DisGeNET | Detail |
| Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocytes in the degenerati... | DisGeNET | Detail |
| Nine and 36 protein spots displayed elevated and suppressed abundance respectively in G93A-SOD1-expr... | DisGeNET | Detail |
| Furthermore, the extent of sumoylation of EAAT2 does not change during the course of ALS in the SOD1... | DisGeNET | Detail |
| DJ-1 changes in G93A-SOD1 transgenic mice: implications for oxidative stress in ALS. | DisGeNET | Detail |
| Protective effects of resveratrol through the up-regulation of SIRT1 expression in the mutant hSOD1-... | DisGeNET | Detail |
| Galectin-1 accumulation in motor axons occurs before the development of ALS-like symptoms and is ass... | DisGeNET | Detail |
| Strains of Saccharomyces cerevisiae that express either the wild type or the amyotrophic lateral scl... | DisGeNET | Detail |
| Furthermore, using an astroglial cell line, primary culture of astrocytes, and tissue samples from G... | DisGeNET | Detail |
| Altogether these findings indicate that G93A-SOD1 expression results in an exacerbated pattern of ne... | DisGeNET | Detail |
| Nine and 36 protein spots displayed elevated and suppressed abundance respectively in G93A-SOD1-expr... | DisGeNET | Detail |
| These findings suggest that intravenous administration of MSCs significantly improves the clinical o... | DisGeNET | Detail |
| The present study was designed to investigate the therapeutic potential of human MSC (hMSC) in the m... | DisGeNET | Detail |
| Exposure to an environmental neurotoxicant hastens the onset of amyotrophic lateral sclerosis-like p... | DisGeNET | Detail |
| Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel expression in the br... | DisGeNET | Detail |
| Consistent with this, we have demonstrated that overexpression of Bcl-2, a protein that inhibits apo... | DisGeNET | Detail |
| Since enhanced iron levels are discussed to participate in oxidative stress and neuronal death, we a... | DisGeNET | Detail |
| Pre-symptomatic detection of chronic motor deficits and genotype prediction in congenic B6.SOD1(G93A... | DisGeNET | Detail |
| We report that the expression of mutant G93A copper/zinc superoxide dismutase (SOD1), associated wit... | DisGeNET | Detail |
| The effect of leukaemia inhibitory factor on SOD1 G93A murine amyotrophic lateral sclerosis. | DisGeNET | Detail |
| Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophi... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| Inhibition of glycogen synthase kinase-3 suppresses the onset of symptoms and disease progression of... | DisGeNET | Detail |
| TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model. | DisGeNET | Detail |
| We have tested the hypothesis that urinary levels of extracellular neurotrophin receptor p75 serve a... | DisGeNET | Detail |
| Transgenic mice that overexpress the G93A mutation of the human Cu-Zn superoxide dismutase 1 gene (h... | DisGeNET | Detail |
| Because peroxisome proliferator-activated receptor gamma (PPARgamma) agonists act as potent anti-inf... | DisGeNET | Detail |
| Astrocyte dysfunction also occurs in human ALS and transgenic rodents expressing mutant human SOD1 p... | DisGeNET | Detail |
| We suggest that the progressive disruption of M1 corticocortical connections resulting from the SOD1... | DisGeNET | Detail |
| We examined the role of MMP-9 in a mouse model of familial ALS and found that lack of MMP-9 increase... | DisGeNET | Detail |
| Adaptive immune neuroprotection in G93A-SOD1 amyotrophic lateral sclerosis mice. | DisGeNET | Detail |
| Downregulation of the potassium chloride cotransporter KCC2 in vulnerable motoneurons in the SOD1-G9... | DisGeNET | Detail |
| G93A SOD1 transgenic mice show an increase in proteasome activity and induction of immuno-proteasome... | DisGeNET | Detail |
| Changes in endocannabinoid receptors and enzymes in the spinal cord of SOD1(G93A) transgenic mice an... | DisGeNET | Detail |
| Here we determined that dysregulated redox stress in ALS mice caused by NADPH oxidases Nox1 and Nox2... | DisGeNET | Detail |
| Therefore, we investigate the influence of the soluble factors released by hADSCs on the GLT1 in pri... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| Time course of preferential motor unit loss in the SOD1 G93A mouse model of amyotrophic lateral scle... | DisGeNET | Detail |
| Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrop... | DisGeNET | Detail |
| We established a transgenic mouse line overexpressing the familial ALS-associated G93A-SOD1 mutation... | DisGeNET | Detail |
| We herein characterised the expression and activity of the glutamate transporter glutamate transport... | DisGeNET | Detail |
| Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolated complex IV defici... | DisGeNET | Detail |
| A major QTL on mouse chromosome 17 resulting in lifespan variability in SOD1-G93A transgenic mouse m... | DisGeNET | Detail |
| We therefore investigated the effects of lithium and VPA cotreatment on the disease symptom onset, s... | DisGeNET | Detail |
| Immunohistochemical analysis of sympathetic involvement in the SOD1-G93A transgenic mouse model of a... | DisGeNET | Detail |
| We detected this interaction both in spinal cord extracts of mutant SOD1(G93A) transgenic mice and i... | DisGeNET | Detail |
| Transgenic mice overexpressing a mutated form of human SOD1 with a Gly93Ala substitution develop pro... | DisGeNET | Detail |
| Amino acids as biomarkers in the SOD1(G93A) mouse model of ALS. | DisGeNET | Detail |
| To gain new mechanistic insights into the role of these receptors in the context of ALS, here we inv... | DisGeNET | Detail |
| However, daily administration of dantrolene to mice overexpressing superoxide dismutase 1 glycine to... | DisGeNET | Detail |
| Adenosine A2A receptors activation facilitates neuromuscular transmission in the pre-symptomatic pha... | DisGeNET | Detail |
| To test the hypothesis that inhibitory interneuron innervation of spinal motoneurons is abnormal in ... | DisGeNET | Detail |
| Depressed excitability and ion currents linked to slow exocytotic fusion pore in chromaffin cells of... | DisGeNET | Detail |
| We show in the SOD1(G93A) mouse model of ALS that recurrent exposure to restraint stress led to an e... | DisGeNET | Detail |
| Furthermore, we also succeeded in mutant SOD1-specific gene suppression in the liver of SOD1(G93A) T... | DisGeNET | Detail |
| Based on the temporal correlation of these impairments with the onset of motor weakness and the appe... | DisGeNET | Detail |
| This finding highlights the utility of the SOD1-G93A transgenic mouse model for preclinical drug stu... | DisGeNET | Detail |
| Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS. | DisGeNET | Detail |
| Proteomic analysis of the transgenic SOD1(G93A) ALS rat model revealed significant up-regulation of ... | DisGeNET | Detail |
| We developed an in vitro disease model with motor neuron-neuroblastoma hybrid cells (VSC4.1) constit... | DisGeNET | Detail |
| Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel expression in the br... | DisGeNET | Detail |
| Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrop... | DisGeNET | Detail |
| Neuroprotective effects of diallyl trisulfide in SOD1-G93A transgenic mouse model of amyotrophic lat... | DisGeNET | Detail |
| Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF2α, and their modula... | DisGeNET | Detail |
| However, no systematic studies focusing on such effects using ALS models other than SOD1(G93A) mice ... | DisGeNET | Detail |
| In an ALS mouse model carrying the human Cu/Zn superoxide dismutase (hSOD1) G93A transgene, ovariect... | DisGeNET | Detail |
| Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show that motor neurons with... | DisGeNET | Detail |
| In the present study, we investigated the time course of microglial (major histocompatibility-II ant... | DisGeNET | Detail |
| Astrocytes expressing the ALS-linked SOD1(G93A) mutation display a decreased mitochondrial respirato... | DisGeNET | Detail |
| In a recent work, we have observed that calcineurin activity is depressed in two models for familial... | DisGeNET | Detail |
| In a recent work, we have observed that calcineurin activity is depressed in two models for familial... | DisGeNET | Detail |
| While there are legitimate concerns about the physiological differences between the rodent and human... | DisGeNET | Detail |
| TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model. | DisGeNET | Detail |
| In this work, we aimed to investigate whether transplanted MN could prevent motor deterioration in t... | DisGeNET | Detail |
| We detected this interaction both in spinal cord extracts of mutant SOD1(G93A) transgenic mice and i... | DisGeNET | Detail |
| Limited effects of glatiramer acetate in the high-copy number hSOD1-G93A mouse model of ALS. | DisGeNET | Detail |
| Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A) transgenic mouse m... | DisGeNET | Detail |
| We therefore studied whether motor neuron disease (MND) in mice expressing the human SOD1 mutant G93... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| The neuroprotective potential of those two genes was further investigated in vivo in the SOD1(G93A) ... | DisGeNET | Detail |
| Therefore, in the present study, we investigated the process of motor neuron degeneration as the dis... | DisGeNET | Detail |
| In the present study, we used the superoxide dismutase 1(SODI)(G93A) transgenic mice as an in vivo m... | DisGeNET | Detail |
| In this study we analyzed the effects of ALS on bone using the well established G93A transgenic mous... | DisGeNET | Detail |
| Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show that motor neurons with... | DisGeNET | Detail |
| To investigate whether GOS could provide neuroprotective effect in ALS, we applied GOS and GOS-rich ... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| Experiments were performed by inducing exocytosis in wild-type (WT) mice, in amphiphysin-I knockout ... | DisGeNET | Detail |
| In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant transgenic mice bef... | DisGeNET | Detail |
| This study attempted to determine the effect of HUCB on SOD1 mice (transgenic B6SJL-TgN(SOD1-G93A)1G... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| We show here that the expression of caspase-11 is upregulated in the spinal cord of superoxide dismu... | DisGeNET | Detail |
| We employed ALS model SOD1(G93A) mice and GlcNAc6ST-1(-/-) mice, which are KS-deficient in the centr... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| Moreover, we demonstrate that upon expression in yeast cells the common ALS-linked mutant Sod1(G93A)... | DisGeNET | Detail |
| Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting neuronal cell loss, ... | DisGeNET | Detail |
| The lack of effect of specific overexpression of IGF-1 in the central nervous system or skeletal mus... | DisGeNET | Detail |
| Ventral roots from SOD1(G93A) ALS mice were analyzed by label-free quantitative mass spectrometry, a... | DisGeNET | Detail |
| In a recent work, we have observed that calcineurin activity is depressed in two models for familial... | DisGeNET | Detail |
| Dose-dependent efficacy of ALS-human mesenchymal stem cells transplantation into cisterna magna in S... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| These results indicate that local complement activation and increased expression of CD88 may contrib... | DisGeNET | Detail |
| Characterization of human sporadic ALS biomarkers in the familial ALS transgenic mSOD1(G93A) mouse m... | DisGeNET | Detail |
| Here, we examine the potential contribution of peripheral axon ensheathing Schwann cells to ALS by c... | DisGeNET | Detail |
| Proteomic analysis of the transgenic SOD1(G93A) ALS rat model revealed significant up-regulation of ... | DisGeNET | Detail |
| Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocytes in the degenerati... | DisGeNET | Detail |
| Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS. | DisGeNET | Detail |
| Taken together, this suggests that reduced levels of VGLUT2 decrease motor neuron degeneration but d... | DisGeNET | Detail |
| The pathogenic SOD1 variants A4V and G93A have been the foci of multiple studies aimed at understand... | DisGeNET | Detail |
| Zinc pre-treatment enhances NMDAR-mediated excitotoxicity in cultured cortical neurons from SOD1(G93... | DisGeNET | Detail |
| A structure activity relationship of a lead scaffold exhibiting neuroprotective activity in a G93A-S... | DisGeNET | Detail |
| In this article, we report a proteomic analysis using a widely used ALS mouse model to identify diff... | DisGeNET | Detail |
| Transgenic mice that express a mutant Cu,Zn-SOD, Gly93 --> Ala (G93A), have been shown to develop... | DisGeNET | Detail |
| We demonstrated that expression of the fully active G93A Cu,Zn superoxide dismutase mutant in neurob... | DisGeNET | Detail |
| The lack of effect of specific overexpression of IGF-1 in the central nervous system or skeletal mus... | DisGeNET | Detail |
| Therefore, we investigate the influence of the soluble factors released by hADSCs on the GLT1 in pri... | DisGeNET | Detail |
| We studied the effect of a high (HEX) and moderate-level exercise (MEX) on body weight, motor perfor... | DisGeNET | Detail |
| We conclude that miR-206 is elevated in the circulation of symptomatic SOD1-G93A mice and possibly i... | DisGeNET | Detail |
| Impairment of nuclear factor-kappaB activation increased glutamate excitotoxicity in a motoneuron-ne... | DisGeNET | Detail |
| We have compared FMO mRNA expression in the control mouse strain C57BL/6J and in a SOD1-mutated (G93... | DisGeNET | Detail |
| A transgenic mouse model of amyotrophic lateral sclerosis was selected based on a phenotype resembli... | DisGeNET | Detail |
| To elucidate the underlying molecular events, we used rapid charge-cooled device imaging to evaluate... | DisGeNET | Detail |
| We examined protein levels of the glutamate transporters GLT-1, GLAST and EAAC1 in the G93A (SOD1) t... | DisGeNET | Detail |
| Transgenic mice carrying the G93A mutation of the superoxide dismutase 1 (SOD1) gene develop a neuro... | DisGeNET | Detail |
| Degeneration of corticospinal and bulbospinal systems in the superoxide dismutase 1(G93A G1H) transg... | DisGeNET | Detail |
| To test whether ALS2 plays a protective role against mutant SOD1-mediated motor neuron degeneration ... | DisGeNET | Detail |
| Transgenic mice overexpressing the human mutated form (G93A) of Cu,Zn-superoxide dismutase (mSOD1) d... | DisGeNET | Detail |
| In the current study, we have examined the therapeutic efficacy of memantine in an ALS mouse model c... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A) transgenic mouse m... | DisGeNET | Detail |
| We developed motor neuron-neuroblastoma hybrid cells that expressed a mutant (G93A) or the wild type... | DisGeNET | Detail |
| Thus, hSOD1-G93A mutation causes both motor and sensory neuropathies, and as such the disease develo... | DisGeNET | Detail |
| To characterize the cellular response to mitochondrial perturbations at the level of gene expression... | DisGeNET | Detail |
| The effect of leukaemia inhibitory factor on SOD1 G93A murine amyotrophic lateral sclerosis. | DisGeNET | Detail |
| Our results indicate that the current density at high GABA concentrations is similar in control, Cu,... | DisGeNET | Detail |
| Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-93 --> Ala (G93A)... | DisGeNET | Detail |
| In the present study, we investigated the time course of microglial (major histocompatibility-II ant... | DisGeNET | Detail |
| This study was undertaken to evaluate the effect of EGCG on ALS model mice with the human G93A mutat... | DisGeNET | Detail |
| The present study provides the first evidence that MnSOD immunoreactivity was increased in the centr... | DisGeNET | Detail |
| In the present study, we injected MSCs into the cerebrospinal fluid of symptomatic hSOD1(G93A) rats,... | DisGeNET | Detail |
| The relevance of identified genes as clinical biomarkers was tested in the peripheral blood transcri... | DisGeNET | Detail |
| Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic... | DisGeNET | Detail |
| In this study, we demonstrate complement system involvement in a rodent model of ALS (human SOD1(G93... | DisGeNET | Detail |
| Here we use a SOD1-ALS mouse model (low-copy Gurney G93A-SOD1 mouse) to show that motor neurons with... | DisGeNET | Detail |
| In order to investigate the effect of various calcium modulators and the SOD-1 mutation on neuronal ... | DisGeNET | Detail |
| We studied the behaviors of spinal neurons using an in vitro preparation of the sacral cord from the... | DisGeNET | Detail |
| We examined protein levels of the glutamate transporters GLT-1, GLAST and EAAC1 in the G93A (SOD1) t... | DisGeNET | Detail |
| To test a new therapeutic strategy for ALS, we examined the effect of caspase inhibition in transgen... | DisGeNET | Detail |
| In the present study, we demonstrate an age-dependent increase in Bcl-xL and Ets-2 immunoreactivity ... | DisGeNET | Detail |
| Galectin-1 accumulation in motor axons occurs before the development of ALS-like symptoms and is ass... | DisGeNET | Detail |
| Increased number and differentiation of neural precursor cells in the brainstem of superoxide dismut... | DisGeNET | Detail |
| Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A ... | DisGeNET | Detail |
| Progressive loss of a glial potassium channel (KCNJ10) in the spinal cord of the SOD1 (G93A) transge... | DisGeNET | Detail |
| Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotro... | DisGeNET | Detail |
| Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dis... | DisGeNET | Detail |
| In this study, we analyzed the spatiotemporal alterations of phospholipid composition in the spinal ... | DisGeNET | Detail |
| To test whether ALS2 plays a protective role against mutant SOD1-mediated motor neuron degeneration ... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| To better understand the autonomic involvement in ALS we measured autonomic functions in transgenic ... | DisGeNET | Detail |
| Erythropoietin modulates the immune-inflammatory response of a SOD1(G93A) transgenic mouse model of ... | DisGeNET | Detail |
| Transgenic mice carrying the G93A mutation of the superoxide dismutase 1 (SOD1) gene develop a neuro... | DisGeNET | Detail |
| Functional over-load saves motor units in the SOD1-G93A transgenic mouse model of amyotrophic latera... | DisGeNET | Detail |
| Metformin treatment has no beneficial effect in a dose-response survival study in the SOD1(G93A) mou... | DisGeNET | Detail |
| D-serine is increased two-fold in spinal cords of G93A Cu,Zn-superoxide dismutase (SOD1) mice--the s... | DisGeNET | Detail |
| Even though the single transgenic hHsp27 mice showed protection against spinal cord ischemia, the do... | DisGeNET | Detail |
| Gonadectomy and dehydroepiandrosterone (DHEA) do not modulate disease progression in the G93A mutant... | DisGeNET | Detail |
| Changes in Wnt5a and Fzd2 expression in the spinal cord of SOD1(G93A) transgenic mice (ALS), SOD1(G9... | DisGeNET | Detail |
| We determined the expression of Wnt3a, β-catenin, and Cyclin D1 in the adult spinal cord of SOD1(G93... | DisGeNET | Detail |
| Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A) transgenic mouse m... | DisGeNET | Detail |
| Using 1H-15N HSQC NMR spectroscopy, we have analyzed hydrogen exchange at the amide groups of wild-t... | DisGeNET | Detail |
| We then investigated whether SA4503 suppressed the progression of ALS in an SOD1(G93A) ALS mouse mod... | DisGeNET | Detail |
| Behavioural and anatomical effects of systemically administered leukemia inhibitory factor in the SO... | DisGeNET | Detail |
| In contrast, CCS/G93A SOD1 mouse spinal cord showed no reduction in levels of selected subunits from... | DisGeNET | Detail |
| The present study thus provides direct evidence of activated UPR in motor neurons which overexpress ... | DisGeNET | Detail |
| To understand better the role of these mutations in the pathophysiology of FALS we have compared the... | DisGeNET | Detail |
| Although up-regulation of caspase-12 has been reported in G93A SOD1 transgenic mice, it is controver... | DisGeNET | Detail |
| Since the mechanism whereby mutations in SOD1 lead to MND remains enigmatic, we asked whether NF inc... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| We here investigated the level of activation of the Ca(2+)-dependent protease calpain-1 in spinal co... | DisGeNET | Detail |
| We report that the expression of mutant G93A copper/zinc superoxide dismutase (SOD1), associated wit... | DisGeNET | Detail |
| Acute glial activation by stab injuries does not lead to overt damage or motor neuron degeneration i... | DisGeNET | Detail |
| Upregulation of amyloid beta (A4) precursor protein (APP) in muscle fibres coincides with symptom on... | DisGeNET | Detail |
| These results indicate that local complement activation and increased expression of CD88 may contrib... | DisGeNET | Detail |
| Background and gender effects on survival in the TgN(SOD1-G93A)1Gur mouse model of ALS. | DisGeNET | Detail |
| Gastric bleeding was not induced by AAD-2004 even at a dose 400-fold higher than that required to ob... | DisGeNET | Detail |
| We used SOD1(G93A) mice as a model, which exhibit the motor neuron-specific neurodegenerative diseas... | DisGeNET | Detail |
| Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF2α, and their modula... | DisGeNET | Detail |
| On the relation of oxidative stress to neuroinflammation: lessons learned from the G93A-SOD1 mouse m... | DisGeNET | Detail |
| To understand better the role of these mutations in the pathophysiology of FALS we have compared the... | DisGeNET | Detail |
| Here we determined that dysregulated redox stress in ALS mice caused by NADPH oxidases Nox1 and Nox2... | DisGeNET | Detail |
| Treatments to rescue motor neurons according to dying-forward model of motor neuron pathology in ALS... | DisGeNET | Detail |
| Long-term (10-11 weeks) transplantation of hNT Neurons into the L(4)-L(5) segments of the ventral ho... | DisGeNET | Detail |
| Transgenic mice overexpressing a mutant human SOD1 gene (G93A-SOD1) were used to examine the mitocho... | DisGeNET | Detail |
| Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS. | DisGeNET | Detail |
| Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrop... | DisGeNET | Detail |
| Neuroprotective effects of JGK-263 in transgenic SOD1-G93A mice of amyotrophic lateral sclerosis. | DisGeNET | Detail |
| In this work we evaluated the locomotor performance of the SOD1(G93A) mouse model of ALS using compu... | DisGeNET | Detail |
| Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A) in neuroblastoma ce... | DisGeNET | Detail |
| Arylsulfanyl pyrazolones block mutant SOD1-G93A aggregation. Potential application for the treatment... | DisGeNET | Detail |
| Using an ALS mouse model carrying a high copy number of a mutant human superoxide dismutase-1 (SOD1)... | DisGeNET | Detail |
| The finding of concomitant dysfunction of upper and lower motoneurons contributes to the validation ... | DisGeNET | Detail |
| The human G93A-SOD1 mutation in a pre-symptomatic rat model of amyotrophic lateral sclerosis increas... | DisGeNET | Detail |
| Here we determined that dysregulated redox stress in ALS mice caused by NADPH oxidases Nox1 and Nox2... | DisGeNET | Detail |
| Both Nrf2 and HO-1 levels were increased and co-localized with reactive astrocytes in the degenerati... | DisGeNET | Detail |
| We employed ALS model SOD1(G93A) mice and GlcNAc6ST-1(-/-) mice, which are KS-deficient in the centr... | DisGeNET | Detail |
| ) delivery of recombinant vascular endothelial growth factor (Vegf) in a SOD1(G93A) rat model of ALS... | DisGeNET | Detail |
| G93A Cu/Zn superoxide dismutase (SOD1), a human mutant SOD1 associated with familial amyotrophic lat... | DisGeNET | Detail |
| Specifically, we observe aberrant endogenous pulsatile GH secretion, reduced pituitary GH content, a... | DisGeNET | Detail |
| The purpose of this study was to determine structural changes which trigger the onset and progressio... | DisGeNET | Detail |
| Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 tra... | DisGeNET | Detail |
| We suggest that the progressive disruption of M1 corticocortical connections resulting from the SOD1... | DisGeNET | Detail |
| Our study provides a new mechanistic link for hSOD1(G93A)-mediated impairment of LE transport to aut... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| We established a transgenic mouse line overexpressing the familial ALS-associated G93A-SOD1 mutation... | DisGeNET | Detail |
| We developed an in vitro disease model with motor neuron-neuroblastoma hybrid cells (VSC4.1) constit... | DisGeNET | Detail |
| The present study provides the first in vivo evidence that alpha-synuclein immunoreactivity was incr... | DisGeNET | Detail |
| In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mo... | DisGeNET | Detail |
| Analysis of motor-evoked potentials recorded from the thoracic spinal cord of symptomatic SOD1(G93A)... | DisGeNET | Detail |
| Here, we demonstrate that the interferon (IFN) signaling pathway is activated in human SOD1(G93A) tr... | DisGeNET | Detail |
| We reported that Cu,Zn-SOD can catalyze free radical generation and a FALS mutant, G93A, exhibits an... | DisGeNET | Detail |
| We then analyzed molecular changes in microglia during neurodegenerative disease activation using th... | DisGeNET | Detail |
| PACAP signaling exerts opposing effects on neuroprotection and neuroinflammation during disease prog... | DisGeNET | Detail |
| To test allele-specific silencing in vivo, we made transgenic mice that express an shRNA against mut... | DisGeNET | Detail |
| We developed motor neuron-neuroblastoma hybrid cells that expressed a mutant (G93A) or the wild type... | DisGeNET | Detail |
| Our data indicate that the clinically relevant SOD1 G93A mutation, which leads to amyotrophic latera... | DisGeNET | Detail |
| Excitability properties of mouse motor axons in the mutant SOD1(G93A) model of amyotrophic lateral s... | DisGeNET | Detail |
| Presymptomatic treatment with acetylcholinesterase antisense oligonucleotides prolongs survival in A... | DisGeNET | Detail |
| Therefore, NSC-34/hSOD1(G93A) cells are a suitable cell model to study GA dysfunction in ALS. | DisGeNET | Detail |
| To characterize the cellular response to mitochondrial perturbations at the level of gene expression... | DisGeNET | Detail |
| The transgenic SOD1(G93A) mouse is a model of human amyotrophic lateral sclerosis (ALS) and recapitu... | DisGeNET | Detail |
| Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitoc... | DisGeNET | Detail |
| We demonstrated that expression of the fully active G93A Cu,Zn superoxide dismutase mutant in neurob... | DisGeNET | Detail |
| Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolated complex IV defici... | DisGeNET | Detail |
| These findings suggest that the expression of the SOD1(G93A G1H) mutant protein results in a disease... | DisGeNET | Detail |
| Since glutamate toxicity has been proposed as a mechanism for the degeneration of motor neurons in a... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| To assess molecular pathological effects of the anti-inflammatory peroxisome proliferator-activated ... | DisGeNET | Detail |
| G93A SOD1 transgenic mice overexpressing CCS protein develop an accelerated disease course that is a... | DisGeNET | Detail |
| In this study, we demonstrated that the level of 5-MTHF is significantly decreased in the plasma, sp... | DisGeNET | Detail |
| These findings provide the first evidence of SP having a physiological and protective role in the G9... | DisGeNET | Detail |
| Transgenic rats expressing a mutated form of the human Cu/Zn superoxide dismutase (hSOD1(G93A)) deve... | DisGeNET | Detail |
| Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A ... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop a delayed and progr... | DisGeNET | Detail |
| Altered expression of astroglial glutamate transporters accompanied by reduced capacity for spinal c... | DisGeNET | Detail |
| Cross-breeding of SOD1(G93A) ALS mice with asic1a-deficient mice delayed the onset and progression o... | DisGeNET | Detail |
| Low levels of SMN also occur in models of amyotrophic lateral sclerosis (ALS) caused by mutant super... | DisGeNET | Detail |
| Relationship between neuropathology and disease progression in the SOD1(G93A) ALS mouse. | DisGeNET | Detail |
| In transgenic mice carrying the G93A human mutation of Cu/Zn superoxide dismutase (SOD1), which prov... | DisGeNET | Detail |
| Experiments were performed by inducing exocytosis in wild-type (WT) mice, in amphiphysin-I knockout ... | DisGeNET | Detail |
| Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop a delayed and progr... | DisGeNET | Detail |
| Early changes of neuromuscular transmission in the SOD1(G93A) mice model of ALS start long before mo... | DisGeNET | Detail |
| G93A SOD1 alters cell cycle in a cellular model of Amyotrophic Lateral Sclerosis. | DisGeNET | Detail |
| Increased ceramide, glucosylceramide, GM3, and hexosaminidase activity were also found in SOD1(G93A)... | DisGeNET | Detail |
| The present study provides the first evidence that MnSOD immunoreactivity was increased in the centr... | DisGeNET | Detail |
| We report that symptomatic male hSOD1(G93A) transgenic mice exhibit a deficiency in GH secretion sim... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| The SOD1 transgene in the G93A mouse model of amyotrophic lateral sclerosis lies on distal mouse chr... | DisGeNET | Detail |
| Furthermore, using an astroglial cell line, primary culture of astrocytes, and tissue samples from G... | DisGeNET | Detail |
| Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrop... | DisGeNET | Detail |
| Protective effects of resveratrol through the up-regulation of SIRT1 expression in the mutant hSOD1-... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| We suggest that the progressive disruption of M1 corticocortical connections resulting from the SOD1... | DisGeNET | Detail |
| Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-93 --> Ala (G93A)... | DisGeNET | Detail |
| Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein. | DisGeNET | Detail |
| Overexpression of a familial mutant form of superoxide dismutase 1 (SOD1) (G93A) in neuroblastoma ce... | DisGeNET | Detail |
| Here, we examine whether prefrontal cortex neuronal abnormalities and related cognitive impairments ... | DisGeNET | Detail |
| G93A SOD1 expression in motor neurons of C. elegans results in characteristic alterations of ALS. | DisGeNET | Detail |
| Protein interactions of the Amyotrophic Lateral Sclerosis (ALS)-linked copper-zinc superoxide dismut... | DisGeNET | Detail |
| Lentivirus and adeno-associated virus have been used to knockdown levels of mutated superoxide dismu... | DisGeNET | Detail |
| delivery of Ara-C accelerated disease progression in SOD1(G93A) mouse model of ALS. | DisGeNET | Detail |
| We recently established that the SOD1-G93A transgenic mouse is a suitable model for oral-stage dysph... | DisGeNET | Detail |
| By placing running wheels in each mouse's cage to measure their exercise activity, we show that pres... | DisGeNET | Detail |
| We show in the SOD1(G93A) mouse model of ALS that recurrent exposure to restraint stress led to an e... | DisGeNET | Detail |
| Genetic biomarkers for ALS disease in transgenic SOD1(G93A) mice. | DisGeNET | Detail |
| To comparatively evaluate the therapeutic potentials of human bone marrow-derived mesodermal stromal... | DisGeNET | Detail |
| We therefore compared onset and progression of disease symptoms in female and male transgenic ALS mi... | DisGeNET | Detail |
| In the present study, we demonstrate an age-dependent increase in Bcl-xL and Ets-2 immunoreactivity ... | DisGeNET | Detail |
| In addition, ZnT3 and ZnT6 protein levels were not altered in SOD1 (G93A) mutant transgenic mice bef... | DisGeNET | Detail |
| Recently we studied glycine receptors (GlyRs) in motoneurons in an ALS mouse model expressing a muta... | DisGeNET | Detail |
| Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the ... | DisGeNET | Detail |
| Altered expression of DJ-1 and PINK1 in sporadic ALS and in the SOD1(G93A) ALS mouse model. | DisGeNET | Detail |
| Crossing the daf-2(e1370) mutation into the G93A SOD1 mutant worms significantly ameliorated the mot... | DisGeNET | Detail |
| Specifically, we observe aberrant endogenous pulsatile GH secretion, reduced pituitary GH content, a... | DisGeNET | Detail |
| Female SOD1(G93A) mice housed with environmental enrichment were found to reach overt end-stage dise... | DisGeNET | Detail |
| Here, we tested the potential neuroprotective effects of UCP2 and its ability to modulate mitochondr... | DisGeNET | Detail |
| Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotr... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| Recombinant human erythropoietin suppresses symptom onset and progression of G93A-SOD1 mouse model o... | DisGeNET | Detail |
| In parallel, MSCs derived from the bone marrow of a transgenic rat model of familial ALS (hSOD1(G93A... | DisGeNET | Detail |
| Immunohistochemical study on the distribution of insulin-like growth factor-binding protein 4 in the... | DisGeNET | Detail |
| Results indicate that while whisking still occurs in SOD1(G93A) mice and is relatively resistant to ... | DisGeNET | Detail |
| The basal glutamate efflux from spinal cord nerve terminals of mice-expressing human soluble superox... | DisGeNET | Detail |
| Functional neural stem cell isolation from brains of adult mutant SOD1 (SOD1(G93A)) transgenic amyot... | DisGeNET | Detail |
| Abnormal medial prefrontal cortex connectivity and defective fear extinction in the presymptomatic G... | DisGeNET | Detail |
| Identification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic l... | DisGeNET | Detail |
| We report here that a familial mouse model (transgenic mice over-expressing the G93A mutation of the... | DisGeNET | Detail |
| Fast-twitch and slow-twitch muscles from symptomatic SOD1-G93A mice (age 90 days) and their control ... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| The work is a continuation of studies on tau expression and alternative splicing in the central nerv... | DisGeNET | Detail |
| The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord syna... | DisGeNET | Detail |
| The precise mechanism by which this receptor drives the apoptotic process, both in murine SOD1-G93A ... | DisGeNET | Detail |
| As proof of concept, we use the IS paradigm to express murine Interleukin (IL)-10 in the spinal cord... | DisGeNET | Detail |
| Taken together, this suggests that reduced levels of VGLUT2 decrease motor neuron degeneration but d... | DisGeNET | Detail |
| Recently, we reported that a subpopulation of spinal cord astrocytes degenerates in the microenviron... | DisGeNET | Detail |
| The superoxide dismutase1 (SOD1) G93A mutation does not promote neuronal injury after focal brain is... | DisGeNET | Detail |
| Loss of a single functional Sil1 allele in an ALS mouse model (SOD1-G93A) enhanced ER stress and exa... | DisGeNET | Detail |
| The human G93A-SOD1 mutation in a pre-symptomatic rat model of amyotrophic lateral sclerosis increas... | DisGeNET | Detail |
| By combining immuno-spin trapping and molecular magnetic resonance imaging, in vivo trapped radical ... | DisGeNET | Detail |
| Autophagy activation and neuroprotection by progesterone in the G93A-SOD1 transgenic mouse model of ... | DisGeNET | Detail |
| These findings provide the first evidence of SP having a physiological and protective role in the G9... | DisGeNET | Detail |
| We studied the release of [3H]d-aspartate ([3H]d-ASP) and endogenous glutamate evoked by glycine (GL... | DisGeNET | Detail |
| Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1)... | DisGeNET | Detail |
| Although viral delivery of IGF-I has shown therapeutic efficacy in the SOD1(G93A) mouse model of ALS... | DisGeNET | Detail |
| Point mutations such as G93A and A4V in the human Cu/Zn-superoxide dismutase gene (hSOD1) cause fami... | DisGeNET | Detail |
| We next applied CME repeatedly to living Wld(S) mice and to SOD1(G93A) mice, an animal model of moto... | DisGeNET | Detail |
| Our previous study showed that glycinergic innervation of spinal motoneurons is deficient in an ALS ... | DisGeNET | Detail |
| Three ALS mice [(SOD1-G93A)1Gur] and three controls [(SOD-1)2Gur] were obtained from The Jackson Lab... | DisGeNET | Detail |
| To determine where and when the pathological changes of motor neuron disease begins, we performed a ... | DisGeNET | Detail |
| Furthermore, selective death of embryonal spinal motor neurons from G93A-SOD1 transgenic mice is ind... | DisGeNET | Detail |
| G93A SOD1 transgenic mice overexpressing CCS protein develop an accelerated disease course that is a... | DisGeNET | Detail |
| We demonstrate that transient genetic manipulation of zebrafish to express G93A-SOD1, a mutation ass... | DisGeNET | Detail |
| Effect of genetic background on onset and disease progression in the SOD1-G93A model of amyotrophic ... | DisGeNET | Detail |
| To evaluate the therapeutic potential of human neural progenitor cells (hNPs) in amyotrophic lateral... | DisGeNET | Detail |
| Collectively, these results establish that CCS/G93A SOD1 mice manifest an isolated complex IV defici... | DisGeNET | Detail |
| Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-sup... | DisGeNET | Detail |
| To clarify the biological significance of the interaction of the redox system (Prx2/GPx1) with SOD1 ... | DisGeNET | Detail |
| The dietary exposure to EPA (300 mg/kg/day) in a well-established mouse model of ALS expressing the ... | DisGeNET | Detail |
| To clarify the biological significance of the interaction of the redox system (Prx2/GPx1) with SOD1 ... | DisGeNET | Detail |
| The paper by Butterfield and colleagues reporting the use of redox proteomics to identify oxidativel... | DisGeNET | Detail |
| Disease progression of human SOD1 (G93A) transgenic ALS model rats. | DisGeNET | Detail |
| Measuring early pre-symptomatic changes in locomotion of SOD1-G93A rats--a rodent model of amyotroph... | DisGeNET | Detail |
| These results show that in the SOD1(G93A) model of neurodegenerative diseases, the concentration of ... | DisGeNET | Detail |
| In this study we have analysed the effect of Monocyte Chemoattractant Protein-1 (MCP-1), a chemokine... | DisGeNET | Detail |
| Our results demonstrated that caprylic triglyceride alleviates ALS-type motor impairment through res... | DisGeNET | Detail |
| Innate and adaptive immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)(G93A)... | DisGeNET | Detail |
| We recently established that the SOD1-G93A transgenic mouse is a suitable model for oral-stage dysph... | DisGeNET | Detail |
| Previously, we established that facial MN (FMN) survival levels in the SOD1(G93A) transgenic mouse m... | DisGeNET | Detail |
| Furthermore, the extent of sumoylation of EAAT2 does not change during the course of ALS in the SOD1... | DisGeNET | Detail |
| We report that the expression of mutant G93A copper/zinc superoxide dismutase (SOD1), associated wit... | DisGeNET | Detail |
| Behavioural and anatomical effects of systemically administered leukemia inhibitory factor in the SO... | DisGeNET | Detail |
| To gain insights on the significance of BIs we asked whether neurons with BIs had an intact or fragm... | DisGeNET | Detail |
| Erythropoietin modulates the immune-inflammatory response of a SOD1(G93A) transgenic mouse model of ... | DisGeNET | Detail |
| Vacuolization correlates with spin-spin relaxation time in motor brainstem nuclei and behavioural te... | DisGeNET | Detail |
| In addition, we have recently reported elevated heart rate and blood pressure in transgenic (TG) mic... | DisGeNET | Detail |
| In mice that express the mutant human gene Cu/Zn superoxide dismutase G93A (SOD1), and therefore sim... | DisGeNET | Detail |
| In this study, we examined the regulation of glutamate transporters by type 5 metabotropic glutamate... | DisGeNET | Detail |
| Glycoprotein nonmetastatic melanoma protein B (GPNMB) was identified as an ALS-related factor using ... | DisGeNET | Detail |
| Investigation of the G93A-SOD1 mouse model for ALS revealed increased message and protein levels of ... | DisGeNET | Detail |
| Early vulnerability to ischemia/reperfusion injury in motor terminals innervating fast muscles of SO... | DisGeNET | Detail |
| Mice over-expressing the mutant human G93A-SOD1 are widely used as an animal model of amyotrophic la... | DisGeNET | Detail |
| Because peroxisome proliferator-activated receptor gamma (PPARgamma) agonists act as potent anti-inf... | DisGeNET | Detail |
| Mice bearing the mutated gene for Cu/Zn superoxide dismutase (G93A) are a good model for human amyot... | DisGeNET | Detail |
| Transgenic mice expressing a mutated (G93A) human Cu/Zn superoxide dismutase (SOD1) develop motor ne... | DisGeNET | Detail |
| After identification of the clinical onset in each female G93A mutant SOD1 transgenic mouse, we intr... | DisGeNET | Detail |
| In order to investigate the effect of various calcium modulators and the SOD-1 mutation on neuronal ... | DisGeNET | Detail |
| Here we report that a single injection of a VEGF-expressing lentiviral vector into various muscles d... | DisGeNET | Detail |
| Progressive loss of a glial potassium channel (KCNJ10) in the spinal cord of the SOD1 (G93A) transge... | DisGeNET | Detail |
| Impairment of nuclear factor-kappaB activation increased glutamate excitotoxicity in a motoneuron-ne... | DisGeNET | Detail |
| Here we investigated the expression of XBP1 and ATF6α and phosphorylation of eIF2α, and their modula... | DisGeNET | Detail |
| In superoxide dismutase 1(G93A) transgenic mouse model of ALS, AAD-2004 blocked free radical product... | DisGeNET | Detail |
| Although viral delivery of IGF-I has shown therapeutic efficacy in the SOD1(G93A) mouse model of ALS... | DisGeNET | Detail |
| Mice engineered to express a transgene encoding a human Cu/Zn superoxide dismutase (SOD1) with a Gly... | DisGeNET | Detail |
| In the superoxide dismutase 1 (SOD1)-G93A mouse model of amyotrophic lateral sclerosis (ALS), skelet... | DisGeNET | Detail |
| We characterized synaptosomal glutamate transport activity in a recently developed transgenic rat mo... | DisGeNET | Detail |
Overlapped Transcript Coordinates
| Gene | Transcript ID | Exon Number | Chromosome | Start | Stop | Type | Amino Mutation | Transcript Position | Links |
|---|
Overlapped Transcript
| Gene | Transcript ID | Chromosome | Start | Stop | Links |
|---|
- Gene
- -
- dbSNP
- rs121912438 dbSNP
- Genome
- hg38
- Position
- chr21:31,667,299-31,667,299
- Variant Type
- snv
- Reference Allele
- G
- Alternative Allele
- C
Genome browser