MutL homolog 1(MLH1) is a protein in humans that is encoded by the MLH1 gene. MLH1 protein is one component of a system of seven DNA mismatch repair proteins that coordinate and work in sequential steps to initiate repair of DNA mismatches in humans. The MLH1 gene is often mutated in hereditary nonpolyposis colon cancer (HNPCC). It also plays a role in meiotic recombination. Defects in mismatch repair are found in around 13% of colorectal cancers and are much more frequently due to deficiency of MLH1 than deficiencies of other DNA mismatch repair proteins.
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