COL6A2 (Collagen alpha-2(VI) chain) variants and mutations

COL6A2 (also known as Collagen alpha-2(VI) chain) is a human protein-coding gene encoding a collagen alpha-2(VI) chain protein. It assembles with other collagen VI chains into extracellular microfibrils that support muscle and connective-tissue integrity. Dominant or recessive pathogenic variants cause collagen VI-related muscular dystrophy and myopathy across a broad severity spectrum. This analysis covers 2,058 COL6A2 variants and mutations. Of these, 84% have computational variant effect predictions. Disease context includes Ullrich congenital muscular dystrophy 1B, Bethlem myopathy, and Congenital muscular dystrophy, Ullrich type. Example COL6A2 variants include M1?, M1I, and L2P.

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 COL6A2 variants

Examples include M1?, M1I, L2P, L2V, Q3R, G4R, G4G, T5I. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.