MLH3 is another DNA mismatch repair gene, and autosomal recessive colorectal polyposis is linked to biallelic GPVs (e.g., p.S1188X) in MLH3. In an initial report involving 4 families and 5 cases, patients were diagnosed at a relatively older age (48–52 years), with 1–200 colorectal adenomatous polyps, and some had developed malignancies such as CRC and breast cancer [68]. The gene discussed is MLH3; the disease is breast cancer.