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EA2

Chr 19AD

calcium voltage-gated channel subunit alpha1 A

Also known as: APCA, BI, CACNL1A4, CAV2.1, DEE42, EA2, EIEE42, FHM

The alpha-1A subunit forms the pore of voltage-dependent calcium channels and directs calcium entry into neurons, which is essential for neurotransmitter release and muscle contraction. Mutations cause episodic ataxia type 2 through autosomal dominant inheritance, typically by disrupting normal calcium channel function in cerebellar and other neuronal tissues. CAG repeat expansions in the coding region (21-33 repeats vs normal 4-18) cause spinocerebellar ataxia 6 by creating a toxic polyglutamine tract.

OMIMResearchSummary from RefSeq, OMIM
AD1 OMIM phenotype
Clinical SummaryEA2
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/EA2?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EA2 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
The episodic ataxias.
Graves TD et al.·Handb Clin Neurol
2024Review
Episodic ataxias.
Jen JC et al.·Handb Clin Neurol
2018Review
Episodic ataxia type 2 (EA2) with interictal myokymia and focal dystonia.
Nielsen EN et al.·Cold Spring Harb Mol Case Stud
2022
Top 5 results · since 2015Search PubMed ↗