SCN5A (Q14524) variants and mutations

SCN5A (also known as Q14524) is a human protein-coding gene encoding a sodium channel protein type 5 subunit alpha protein. Its rapid inward sodium current drives the upstroke of the cardiac action potential and enables fast electrical conduction through atrial, ventricular, and conduction-system tissue. Pathogenic variants can cause long-QT syndrome type 3, Brugada syndrome, conduction disease, and overlapping arrhythmia phenotypes. This analysis covers 2,486 SCN5A variants and mutations. Of these, 63% have computational variant effect predictions. Disease context includes Brugada syndrome 1, long QT syndrome 3, and progressive familial heart block, type 1A. Example SCN5A variants include M1I, M1L, and M1T.

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, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable SCN5A variants

Examples include M1I, M1L, M1T, N3D, N3S, P7L, R8Q, R8W. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.