CFTR (P13569) variants and mutations

CFTR (also known as P13569) is a human protein-coding gene encoding a cystic fibrosis transmembrane conductance regulator protein. An epithelial chloride channel and regulator of salt and water movement across cell surfaces. Its activity helps keep airway, intestinal, and other epithelial fluids balanced, while CFTR disruption causes cystic fibrosis and related disorders. This analysis covers 3,261 CFTR variants and mutations. Of these, 96% have computational variant effect predictions. Disease context includes cystic fibrosis, congenital bilateral aplasia of vas deferens from CFTR mutation, and hereditary chronic pancreatitis. Example CFTR variants include M1I, M1K, and M1R.

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 CFTR variants

Examples include M1I, M1K, M1R, M1T, M1V, Q2*, Q2P, Q2Q. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.