Annotation Detail

Information
Associated Genes
OPA1
Associated Variants
OPA1 p.Val940GlyfsTer3 (p.V940Gfs*3) ( ENST00000361150.6, ENST00000361510.8, ENST00000361715.6, ENST00000361828.7, ENST00000361908.8, ENST00000392436.7, ENST00000392437.6, ENST00000643329.1, ENST00000645553.1, ENST00000646793.1 )
OPA1 p.Val940GlyfsTer3 (p.V940Gfs*3) ( ENST00000361150.6, ENST00000361510.8, ENST00000361715.6, ENST00000361828.7, ENST00000361908.8, ENST00000392436.7, ENST00000392437.6, ENST00000643329.1, ENST00000645553.1, ENST00000646793.1 )
Associated Disease
Autosomal dominant optic atrophy classic form Optic atrophy with or without deafness, ophthalmoplegia, myopathy, ataxia, and neuropathy Glaucoma, normal tension, susceptibility to Abortive cerebellar ataxia Mitochondrial DNA depletion syndrome 14 (cardioencephalomyopathic type)
Source Database
ClinVar
Description
NM_130837.3(OPA1):c.2873_2876del AND multiple conditions
ClinVar Allele ID
20121
ClinVar RefSeq Alternation Syntax
NM_130837.3:c.2873_2876del
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2021-07-22
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV002490322
ClinVar Disease
Mitochondrial DNA depletion syndrome 14 (cardioencephalomyopathic type)
ClinVar Disease
Glaucoma, normal tension, susceptibility to
ClinVar Disease
Optic atrophy with or without deafness, ophthalmoplegia, myopathy, ataxia, and neuropathy
ClinVar Disease
Autosomal dominant optic atrophy classic form
ClinVar Disease
Abortive cerebellar ataxia
Observed Origin Sample
unknown
Drugs