CYP17A1 (P05093) variants and mutations

CYP17A1 (also known as P05093) is a human protein-coding gene encoding a steroid 17-alpha-hydroxylase/17,20 lyase protein. Its 17-alpha-hydroxylase and 17,20-lyase activities direct adrenal and gonadal steroid synthesis toward glucocorticoids and sex steroids. Biallelic deficiency causes 17-alpha-hydroxylase/17,20-lyase deficiency with hypertension, hypokalemia, and impaired sexual development. This analysis covers 839 CYP17A1 variants and mutations. Of these, 82% have computational variant effect predictions. Disease context includes congenital adrenal hyperplasia due to 17-alpha-hydroxylase deficiency, 46,XY disorder of sex development due to isolated 17,20 lyase deficiency, and congenital adrenal hyperplasia. Example CYP17A1 variants include M1I, M1T, 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, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable CYP17A1 variants

Examples include M1I, M1T, M1V, W2*, E3D, E3K, V5G, V5L. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.