MYH is a gene involved in repairing DNA and is linked to a hereditary cancer syndrome. It is situated on the short arm (p) of chromosome 1 in the 1p34.3-p32.1 region. MYH produces an enzyme that removes adenine bases from mismatched pairs with 8-oxoguanine, which occur during the replication of oxidized DNA. This gene is also referred to as MUTYH.In individuals with multiple colon polyps, around 30% of those with 15 to 100 polyps possess mutations in both copies of the MYH gene. Moreover, about 10% of families suspected of having familial adenomatous polyposis (FAP) but lacking the typical gene mutation actually have biallelic mutations in the MYH gene.MYH syndrome is inherited in an autosomal recessive manner. Most people with the syndrome do not have a long family history of colon polyps or cancer, although siblings may be affected. The condition can lead to a small number or hundreds of polyps in the colon. There is no link between MYH and tumors outside the colon.In conclusion, MYH syndrome is an autosomal recessive variant of colorectal adenomatous polyposis, also known as familial adenomatous polyposis or familial polyposis of the colon and rectum.