rs727502811

This is a variant in the TOR1A gene that changes a arginine to an glutamine.

ClinVar annotation

Pathogenic☆☆☆
6 submitters7 publications

Dystonic disorder; Early-onset generalized limb-onset dystonia; TOR1A-related disorder

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Research that mentions this SNP (1)

ε‐sarcoglycan mutations found in combination with other dystonia gene mutations
FunctionalN=1,264Christine Klein et al.(2002)· Annals of Neurology

High-resolution melting (HRM) analysis of TOR1A Exon 5 in 1,014 subjects with primary dystonia and 250 controls identified only two carriers of the classic Δ GAG deletion and no c.863G>A mutations. HRM demonstrated 100% diagnostic sensitivity and specificity for detecting TOR1A mutations, establishing it as an efficient screening method for dystonia genetic testing.

Traits studied:BlepharospasmCervical dystoniaGeneralized dystoniaMultifocal dystoniaOromandibular dystoniaPrimary dystoniaSegmental dystoniaSpasmodic dysphoniaWriter's cramp

About TOR1A

The protein encoded by this gene is a member of the AAA family of adenosine triphosphatases (ATPases), is related to the Clp protease/heat shock family and is expressed prominently in the substantia nigra pars compacta. Mutations in this gene result in the autosomal dominant disorder, torsion dystonia 1. [provided by RefSeq, Jul 2008]

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Gene information from NCBI Gene. Variant classifications from ClinVar.

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