Annotation Detail

Information
Associated Genes
ATP1A3
Associated Variants
ATP1A3 p.Thr784Ile (p.T784I), ENSG00000285505 p.Thr771Ile (p.T771I) ( ENST00000543770.5, ENST00000545399.6, ENST00000602133.5, ENST00000648268.1 )
ATP1A3 p.Thr784Ile (p.T784I), ENSG00000285505 p.Thr771Ile (p.T771I) ( ENST00000543770.5, ENST00000545399.6, ENST00000602133.5, ENST00000648268.1 )
Associated Disease
Cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss syndrome
Source Database
ClinVar
Description
NM_152296.5(ATP1A3):c.2312C>T (p.Thr771Ile) AND Cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss syndrome
ClinVar Allele ID
964892
ClinVar RefSeq Alternation Syntax
NM_001256213.2:c.2345C>T
ClinVar RefSeq Alternation Syntax
NM_001256214.2:c.2351C>T
ClinVar RefSeq Alternation Syntax
NM_152296.5:c.2312C>T
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2020-03-02
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV001254116
ClinVar Disease
Cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss syndrome
Observed Origin Sample
de novo
Drugs