KRT1 (Keratin, type II cytoskeletal 1) variants and mutations

KRT1 (also known as Keratin, type II cytoskeletal 1) is a human protein-coding gene encoding a keratin, type II cytoskeletal 1 protein. It pairs with keratin 10 to provide mechanical resilience to suprabasal epidermal cells and help maintain the skin barrier. Dominant pathogenic variants cause epidermolytic ichthyosis and related palmoplantar keratoderma phenotypes. This analysis covers 1,162 KRT1 variants and mutations. Of these, 79% have computational variant effect predictions. Disease context includes epidermolytic ichthyosis, diffuse nonepidermolytic palmoplantar keratoderma, and Non-epidermolytic palmoplantar keratoderma. Example KRT1 variants include S2N, R3Q, and Q4E.

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 KRT1 variants

Examples include S2N, R3Q, Q4E, Q4R, F5S, S6N, S7F, S9P. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.