ATXN3

ataxin 3

Summary

Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2016]

Known Variants36 total

rsidPosition (GRCh37)AllelesClassClinVar
rs965239614:92,525,617T/Ccoding sequence variant
rs77491424714:92,530,677T/Cuncertain significance
rs103771026714:92,530,690C/Auncertain significance
rs714361614:92,531,458A/Cintron variant
rs715033314:92,532,013T/Cintron variant
rs801266614:92,532,620C/Tintron variant
rs1258828714:92,536,959T/Cintron variant
rs141805127314:92,537,193A/Glikely benign
rs89362139814:92,537,323C/Tuncertain significance
rs1289535714:92,537,354C/Gbenign
rs1289658314:92,537,379T/Clikely benign
rs1289658814:92,537,387T/Gbenign
rs1289658914:92,537,388T/Cbenign
rs3420768214:92,544,937T/Cintron variant
rs5986468514:92,545,244A/Gintron variant
rs14705833114:92,547,327C/Tuncertain significance
rs14521045914:92,547,336C/Tuncertain significance
rs105074225314:92,547,399G/Tuncertain significance
rs20199216214:92,547,408G/Auncertain significance
rs76754397314:92,548,695T/Cuncertain significance
rs14836677514:92,548,747T/Guncertain significance
rs77406466714:92,548,775G/Auncertain significance
rs254416941414:92,548,776C/Guncertain significance
rs14214880014:92,548,777T/Clikely benign
rs104875514:92,548,785C/Tbenign
rs14167287214:92,549,495C/Tuncertain significance
rs254420999514:92,549,504T/Cuncertain significance
rs1699914114:92,549,586G/Abenign
rs7987351614:92,553,580A/Tintron variant
rs75400784914:92,555,136G/Auncertain significance
rs1780781514:92,556,055T/Cintron variant
rs75487350414:92,559,626G/Cuncertain significance
rs254479474914:92,562,474G/Auncertain significance
rs14450656614:92,563,028G/Alikely benign
rs254516080114:92,572,880T/Guncertain significance
rs381483414:92,572,927G/Aregulatory region variant

Gene information from NCBI Gene. Variant classifications from ClinVar.