RYR1 (Ryanodine receptor 1) variants and mutations

RYR1 (also known as Ryanodine receptor 1) is a human protein-coding gene encoding a ryanodine receptor 1 protein. It releases calcium from the skeletal-muscle sarcoplasmic reticulum when Cav1.1 senses membrane depolarization, directly coupling excitation to contraction. Pathogenic variants cause malignant-hyperthermia susceptibility and a broad spectrum of congenital RYR1-related myopathies. This analysis covers 7,713 RYR1 variants and mutations. Of these, 73% have computational variant effect predictions. Disease context includes congenital multicore myopathy with external ophthalmoplegia, central core myopathy, and Central core disease. Example RYR1 variants include M1I, G2A, and G2D.

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, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable RYR1 variants

Examples include M1I, G2A, G2D, D3N, D3D, A4S, A4T, A4A. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.