MSH6 (DNA mismatch repair protein Msh6) variants and mutations

MSH6 (also known as DNA mismatch repair protein Msh6) is a human protein-coding gene encoding a DNA mismatch repair protein. Together with MSH2, it recognizes single-base mismatches and small insertion-deletion loops during DNA replication and initiates mismatch repair. Germline loss-of-function variants cause Lynch syndrome, while biallelic variants can cause constitutional mismatch-repair deficiency. This analysis covers 7,388 MSH6 variants and mutations. Of these, 56% have computational variant effect predictions. Disease context includes Lynch syndrome, endometrial cancer, and Constitutional mismatch repair deficiency syndrome. Example MSH6 variants include M1I, M1T, and M1V.

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 MSH6 variants

Examples include M1I, M1T, M1V, S2*, S2A, S2L, S2T, S2W. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.