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This article presents an encyclopedia entry about the Turcot syndrome. It refers to a rare inherited neoplastic disease which is characterized by the association of primary malignant tumors of the central nervous system and colon cancers. The clinical characteristics of this syndrome are described. A brief information about the PMS2 gene is presented.

A definition for the term "postmeiotic segregation increased 2" (PMS2) is presented. PMS2 is a protein of 862 aa and 95 kD that is involved in the mismatch repair of DNA. It also interacts with mutL homolog 1 protein. Mutations in PMS2 are associated with familial hereditary nonpolyposis colon cancer. In PMS2, the gene locus maps to 7p22.