COL5A1 (Collagen alpha-1(V) chain) variants and mutations

COL5A1 (also known as Collagen alpha-1(V) chain) is a human protein-coding gene encoding a collagen alpha-1(V) chain protein. It helps nucleate and regulate type I collagen fibril assembly, controlling fibril diameter and connective-tissue architecture. Haploinsufficiency or structural variants are a major cause of classical Ehlers-Danlos syndrome, with skin hyperextensibility, atrophic scarring, and joint hypermobility. This analysis covers 2,788 COL5A1 variants and mutations. Of these, 68% have computational variant effect predictions. Disease context includes Ehlers-Danlos syndrome, classic type, 1, Ehlers-Danlos syndrome, classic type, and fibromuscular dysplasia, multifocal. Example COL5A1 variants include M1I, M1R, and M1V.

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, MaveDB, LitVar.

Notable COL5A1 variants

Examples include M1I, M1R, M1V, D2E, D2N, D2Y, D2G, D2D. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.