COL17A1 (Collagen alpha-1(XVII) chain) variants and mutations

COL17A1 (also known as Collagen alpha-1(XVII) chain) is a human protein-coding gene encoding a collagen alpha-1(XVII) chain protein. It anchors basal keratinocytes to the basement membrane through hemidesmosomes and is essential for stable epidermal adhesion. Biallelic loss-of-function variants cause junctional epidermolysis bullosa, while autoantibodies against the protein cause bullous pemphigoid. This analysis covers 2,118 COL17A1 variants and mutations. Of these, 76% have computational variant effect predictions. Disease context includes epidermolysis bullosa, junctional 4, intermediate, epithelial recurrent erosion dystrophy, and junctional epidermolysis bullosa, non-Herlitz type. Example COL17A1 variants include D2Y, V3I, and T4A.

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, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable COL17A1 variants

Examples include D2Y, V3I, T4A, K6*, R9*, R9Q, G11R, G11V. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.