HSD3B2 (P26439) variants and mutations

HSD3B2 (also known as P26439) is a human protein-coding gene encoding a 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 2 protein. It catalyzes an early essential step in adrenal and gonadal steroid synthesis, converting pregnenolone and related 3-beta-hydroxysteroids into progesterone-class intermediates. Biallelic deficiency causes congenital adrenal hyperplasia with impaired cortisol and aldosterone synthesis and variable disordered sex development. This analysis covers 894 HSD3B2 variants and mutations. Of these, 93% have computational variant effect predictions. Disease context includes congenital adrenal hyperplasia due to 3-beta-hydroxysteroid dehydrogenase defici, congenital adrenal hyperplasia, and adrenal gland disorder. Example HSD3B2 variants include M1L, G2D, and G2S.

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, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable HSD3B2 variants

Examples include M1L, G2D, G2S, G2V, G2C, G2G, W3*, W3C. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.