Germline mutations in BRCA2, ATM, MLH1, PMS2, CHEK2 and HOXB13 have been identified in association with prostate cancer risk [11,21,22], and carriers of germline MSH2 and MSH6 mutations were found to have higher prostate cancer incidence than age-matched controls (4.3% vs. 3%, respectively) [21,23]. Here, MLH1 is linked to prostate cancer.