CACNA1S (Q13698) variants and mutations

CACNA1S (also known as Q13698) is a human protein-coding gene encoding a voltage-dependent L-type calcium channel subunit alpha-1S protein. Its voltage sensing in skeletal-muscle transverse tubules mechanically activates RYR1 and couples membrane depolarization to sarcoplasmic-reticulum calcium release. Pathogenic variants can cause hypokalemic periodic paralysis, malignant-hyperthermia susceptibility, and congenital myopathy. This analysis covers 3,037 CACNA1S variants and mutations. Of these, 70% have computational variant effect predictions. Disease context includes hypokalemic periodic paralysis, type 1, congenital myopathy 18, and malignant hyperthermia of anesthesia. Example CACNA1S variants include M1V, E2*, and S4Y.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable CACNA1S variants

Examples include M1V, E2*, S4Y, S5L, S5P, Q7*, D8V, E9*. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.