EPAS1 (Q99814) variants and mutations

EPAS1 (also known as Q99814) is a human protein-coding gene encoding an endothelial PAS domain-containing protein 1 protein. When stabilized by hypoxia, it activates hypoxia-responsive genes controlling erythropoiesis, angiogenesis, iron metabolism, and cellular adaptation to low oxygen. Activating variants can cause familial erythrocytosis, while somatic dysregulation is central to clear-cell renal carcinoma and some paragangliomas. This analysis covers 1,846 EPAS1 variants and mutations. Of these, 73% have computational variant effect predictions. Disease context includes erythrocytosis, familial, 4, renal cell carcinoma, and clear cell renal carcinoma. Example EPAS1 variants include T2I, T2R, and T2K.

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 EPAS1 variants

Examples include T2I, T2R, T2K, T2T, A3G, A3P, A3T, A3V. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.