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CAPOS

Chr 19

ATPase Na+/K+ transporting subunit alpha 3

Also known as: AHC2, CAPOS, DEE99, DYT12, RDP

The protein is the alpha-3 subunit of Na+/K+-ATPase, an integral membrane enzyme that establishes and maintains sodium and potassium ion gradients across cell membranes, which are essential for nerve and muscle electrical excitability, osmoregulation, and transport of various molecules. Mutations cause CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss), an autosomal dominant disorder with childhood onset affecting the nervous system, vision, and hearing. The gene is associated with episodic neurological symptoms that can be triggered by fever or illness.

GeneReviewsResearchSummary from RefSeq
Clinical SummaryCAPOS
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
📖
GeneReview available — CAPOS
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (3)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/CAPOS?content-type=application/json&expand=1

gnomad: Error: Gene not found

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CAPOS · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC
[Clinical features of CAPOS syndrome caused by maternal ATP1A3 gene variation: a case report].
Gao Y et al.·Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
2024Case report