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Ataxia v1.137 CACNA1A_SCA6_CAG Bryony Thompson Marked STR: CACNA1A_SCA6_CAG as ready
Ataxia v1.137 CACNA1A_SCA6_CAG Bryony Thompson Str: cacna1a_sca6_cag has been classified as Green List (High Evidence).
Ataxia v1.137 CACNA1A_SCA6_CAG Bryony Thompson Classified STR: CACNA1A_SCA6_CAG as Green List (high evidence)
Ataxia v1.137 CACNA1A_SCA6_CAG Bryony Thompson Str: cacna1a_sca6_cag has been classified as Green List (High Evidence).
Ataxia v1.136 CACNA1A_SCA6_CAG Bryony Thompson STR: CACNA1A_SCA6_CAG was added
STR: CACNA1A_SCA6_CAG was added to Ataxia. Sources: Expert List
Mode of inheritance for STR: CACNA1A_SCA6_CAG was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for STR: CACNA1A_SCA6_CAG were set to 20301319; 29325606
Phenotypes for STR: CACNA1A_SCA6_CAG were set to Spinocerebellar ataxia 6 MIM#183086; Episodic ataxia, type 2 MIM#108500
Review for STR: CACNA1A_SCA6_CAG was set to GREEN
STR: CACNA1A_SCA6_CAG was marked as clinically relevant
STR: CACNA1A_SCA6_CAG was marked as current diagnostic
Added comment: NM_023035.2:c.6929_6931CAG[X]
PolyQ expansion alters gene binding, impairs transcription factor function, and is toxic to cells expressing the α1ACT – effects consistent with a loss of function
Normal: ≤18 repeats
Questionable significance: 19 CAG repeats
Full penetrance: ≥20 repeats
Sources: Expert List