CLCN1 (Chloride channel protein 1) variants and mutations

CLCN1 (also known as Chloride channel protein 1) is a human protein-coding gene encoding a chloride channel protein 1 protein. Its chloride conductance stabilizes the resting membrane potential of skeletal muscle and prevents repetitive firing after contraction. Loss-of-function variants cause myotonia congenita, with delayed muscle relaxation and stiffness. This analysis covers 1,686 CLCN1 variants and mutations. Of these, 72% have computational variant effect predictions. Disease context includes myotonia congenita, autosomal recessive, Thomsen and Becker disease, and Myotonia. Example CLCN1 variants include M1?, E2K, and E2A.

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, MaveDB, LitVar.

Notable CLCN1 variants

Examples include M1?, E2K, E2A, p.Gln3 Ser4del, Q3*, Q3Q, S4F, S4P. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.