CACNA1H

Chr 16AD

calcium voltage-gated channel subunit alpha1 H

Also known as: CACNA1HB, Cav3.2, ECA6, EIG6, HALD4

This gene encodes a T-type member of the alpha-1 subunit family, a protein in the voltage-dependent calcium channel complex. Calcium channels mediate the influx of calcium ions into the cell upon membrane polarization and consist of a complex of alpha-1, alpha-2/delta, beta, and gamma subunits in a 1:1:1:1 ratio. The alpha-1 subunit has 24 transmembrane segments and forms the pore through which ions pass into the cell. There are multiple isoforms of each of the proteins in the complex, either encoded by different genes or the result of alternative splicing of transcripts. Alternate transcriptional splice variants, encoding different isoforms, have been characterized for the gene described here. Studies suggest certain mutations in this gene lead to childhood absence epilepsy (CAE). [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

{Epilepsy, childhood absence, susceptibility to, 6}MIM #611942
{Epilepsy, idiopathic generalized, susceptibility to, 6}MIM #611942
Hyperaldosteronism, familial, type IVMIM #617027
AD
2
Active trials
48
Pubs (1 yr)
P/LP submissions
P/LP missense
0.50
LOEUF
Multiple*
Mechanism· predicted
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.50LOEUF
pLI 0.000
Z-score 5.35
OE 0.38 (0.280.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
-2.36Z-score
OE missense 1.17 (1.131.22)
1717 obs / 1462.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.38 (0.280.50)
00.351.4
Missense OE?1.17 (1.131.22)
00.61.4
Synonymous OE?1.65
01.21.6
LoF obs/exp: 32 / 85.3Missense obs/exp: 1717 / 1462.9Syn Z: -13.03

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CACNA1H · protein map & ClinVar variants

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