MSH3, DUC1, DUG, DNA mismatch repair protein Msh3, hMSH3, Divergent upstream protein, DUP, Mismatch repair protein 1, MRP1
The protein encoded by this gene forms a heterodimer with MSH2 to form MutS beta, part of the post-replicative DNA mismatch repair system. MutS beta initiates mismatch repair by binding to a mismatch and then forming a complex with MutL alpha heterodimer. This gene contains a polymorphic 9 bp tandem repeat sequence in the first exon. The repeat is present 6 times in the reference genome sequence and 3-7 repeats have been reported. Defects in this gene are a cause of susceptibility to endometrial cancer. [provided by RefSeq, Mar 2011],disease:Defects in MSH3 are a cause of susceptibility to endometrial cancer [MIM:608089].,function: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.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,sequence caution:Contaminating sequence. Potential poly-A sequence.,similarity:Belongs to the DNA mismatch repair mutS family. MSH3 subfamily.,subunit:Heterodimer consisting of MSH2-MSH3 (MutS beta). Forms a ternary complex with MutL alpha (MLH1-PMS1). Interacts with EXO1.,
