MSH6是抑癌基因，编码蛋白是mutS蛋白同源物6，参与DNA修复中的错配修复过程。MSH6与MSH2异二聚化以形成MutS-α。该复合物识别单碱基对错配和二核苷酸插入-缺失环，启动错配修复（MMR）过程。MSH6和其他MMR基因与遗传性非息肉病结肠癌（HNPCC，也称为Lynch综合征）相关。MSH6的突变表型较为温和，通常引起非典型的HNPCC。MSH6尽管在结肠癌中最常见，但MSH6突变也已在多种其他癌症类型和综合征中报道，包括子宫内膜癌和子宫癌。

The MSH6 (mutS homolog 6) gene encodes the DNA repair mismatch protein MSH6. MSH6 is a tumor suppressor which heterodimerizes with MSH2 to form MutS-alpha. This complex recognizes single base pair mismatches and dinucleotide insertion-deletion loops, initiating the mismatch repair (MMR) process. MSH6 and other MMR genes are most notably implicated in hereditary non-polyposis colon cancer (HNPCC), also known as Lynch syndrome. Loss of function mutations or epigenetic silencing both in the germline and somatic context lead to an increased mutation rate that drives carcinogenesis as well as microsatellite instability (MSI). Although most commonly seen in colon cancer, MSH6 mutations have also been reported in a wide range of other cancer types and syndromes, including endometrial and uterine cancers.

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