ATXN10

Chr 22

ataxin 10

Also known as: ATX10, E46L, HUMEEP, SCA10

This gene encodes a protein that may function in neuron survival, neuron differentiation, and neuritogenesis. These roles may be carried out via activation of the mitogen-activated protein kinase cascade. Expansion of an ATTCT repeat from 9-32 copies to 800-4500 copies in an intronic region of this locus has been associated with spinocerebellar ataxia, type 10. Alternatively spliced transcript variants have been described.[provided by RefSeq, Jul 2016]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpinocerebellar ataxia 10
2
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
0.70
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — ATXN10
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.70LOEUF
pLI 0.000
Z-score 2.69
OE 0.41 (0.250.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.33Z-score
OE missense 1.06 (0.961.17)
270 obs / 255.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.41 (0.250.70)
00.351.4
Missense OE?1.06 (0.961.17)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 10 / 24.3Missense obs/exp: 270 / 255.4Syn Z: 0.04

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ATXN10 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.