PDX1 (P52945) variants and mutations

PDX1 (also known as P52945) is a human protein-coding gene encoding a pancreas/duodenum homeobox protein 1 protein. It directs pancreatic development and later maintains beta-cell identity and insulin transcription. Biallelic severe loss can cause pancreatic agenesis and neonatal diabetes, while heterozygous variants can cause maturity-onset diabetes of the young. This analysis covers 792 PDX1 variants and mutations. Of these, 90% have computational variant effect predictions. Disease context includes pyruvate dehydrogenase E3-binding protein deficiency, hereditary disease, and mitochondrial disease. Example PDX1 variants include N2K, N2D, and N2N.

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, ClinGen, MaveDB, LitVar.

Notable PDX1 variants

Examples include N2K, N2D, N2N, G3A, G3R, G3S, G3V, G3C. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.