MRE11 (P49959) variants and mutations

MRE11 (also known as P49959) is a human protein-coding gene encoding a double-strand break repair protein. It detects and processes DNA double-strand breaks within the MRE11-RAD50-NBN complex and helps activate ATM-dependent checkpoints. Biallelic hypomorphic variants can cause ataxia-telangiectasia-like disorder with chromosome instability and progressive neurologic disease. This analysis covers 1,657 MRE11 variants and mutations. Of these, 72% have computational variant effect predictions. Disease context includes ataxia-telangiectasia-like disorder, hereditary neoplastic syndrome, and Inherited cancer-predisposing syndrome. Example MRE11 variants include M1I, M1V, and S2G.

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 MRE11 variants

Examples include M1I, M1V, S2G, S2R, S2T, T3A, T3I, A4S. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.