POLE (Q07864) variants and mutations

POLE (also known as Q07864) is a human protein-coding gene encoding a DNA polymerase epsilon catalytic subunit A protein. It performs leading-strand DNA synthesis and proofreads newly incorporated bases during replication. Germline exonuclease-domain variants cause polymerase-proofreading-associated polyposis, while somatic proofreading defects create ultramutated tumors with distinctive mutation signatures. This analysis covers 8,115 POLE variants and mutations. Of these, 59% have computational variant effect predictions. Disease context includes colorectal cancer, susceptibility to, 12, intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia conge, and Facial dysmorphism - immunodeficiency - livedo - short stature. Example POLE variants include M1I, M1K, and M1L.

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, MaveDB, LitVar.

Notable POLE variants

Examples include M1I, M1K, M1L, M1R, M1T, M1V, S2C, S2F. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.