Component of the post-replicative DNA mismatch repair system (MMR). Heterodimerizes with MSH2 to form MutS beta which binds to DNA mismatches thereby initiating DNA repair. When bound, the MutS beta heterodimer bends the DNA helix and shields approximately 20 base pairs. MutS beta recognizes large insertion-deletion loops (IDL) up to 13 nucleotides long. After mismatch binding, forms a ternary complex with the MutL alpha heterodimer, which is thought to be responsible for directing the downstream MMR events, including strand discrimination, excision, and resynthesis.
Involvement in disease
Defects in MSH3 are a cause of susceptibility to endometrial cancer (ENDMC) [MIM:608089].
Belongs to the DNA mismatch repair mutS family. MSH3 subfamily.
Phosphorylated upon DNA damage, probably by ATM or ATR.