The MLH1 gene provides instructions for making a protein that plays an essential role in repairing DNA. This protein helps correct errors that are made when DNA is copied in preparation for cell division, a process called DNA replication. The MLH1 protein joins with another protein called PMS2 to form a two-protein complex called a dimer. This complex coordinates the activities of other proteins that are involved in repairing errors made during DNA replication. The section of DNA that contains an error is removed and replaced with a corrected DNA sequence.
The MLH1 gene is one of a set of genes called mismatch repair (MMR) genes. The MLH1 protein can also form a dimer with the MLH3 or PMS1 protein, but the function of these dimers is not well understood.
Other Names
COCA2; FCC2; MLH-1
Related Genetics Topics
- Lynch syndrome — MedlinePlus Genetics
- Constitutional mismatch repair deficiency syndrome — MedlinePlus Genetics
Identifiers
- NCBI Gene: 4292
- OMIM: 120436
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
