[The G/T Binding Protein (GTBP), encoded by the MSH6 gene, forms Mismatch-Binding Factor with MSH2. Associated with colon, endometrial, and stomach cancers, the MSH6 Gene is a cloned gene for Hereditary Non-Polyposis Colon Cancer. GTBP contains a PWWP domain, binds to G/T mismatches, is required for base/base and single-nucleotide insertion-deletion repairs, and increases the proficiency of two-four nucleotide insertion-deletion repair. ADP-bound MSH2/MSH6 binds to mismatched DNA, provoking ADP/ATP exchange and MSH2/MSH6 migration along the DNA backbone. DNA-bound, ATP-bound MSH2/MSH6 activates the repair machinery. MSH6 is part of the multisubunit BASC complex, which may sense abnormal DNA structures and regulate postreplication repair. This association could be a dynamic process changing throughout the cell cycle and within subnuclear domains. (From SWISS-PROT, OMIM, and NCI) ( NCI )]
UMLS (NCI) C0299348 - Amino Acid, Peptide, or Protein
- Biologically Active Substance