PIEZO1 (Piezo-type mechanosensitive ion channel component 1) variants and mutations

PIEZO1 (also known as Piezo-type mechanosensitive ion channel component 1) is a human protein-coding gene encoding a piezo-type mechanosensitive ion channel component 1 protein. The protein forms a mechanically activated, nonselective cation channel that converts membrane tension into an electrical and calcium signal. It contributes to touch, blood-cell volume control, and lymphatic development, and PIEZO1 variants are associated with dehydrated stomatocytosis and lymphatic malformations. This analysis covers 4,518 PIEZO1 variants and mutations. Of these, 99% have computational variant effect predictions. Disease context includes dehydrated hereditary stomatocytosis with or without pseudohyperkalemia and/or p, lymphedema, hereditary, iii, and lymphatic malformation 6. Example PIEZO1 variants include E2G, P3L, and P3S.

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, PharmGKB, MaveDB, LitVar.

Notable PIEZO1 variants

Examples include E2G, P3L, P3S, H4L, H4Q, H4R, H4Y, V5E. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.