DNA mismatch repair in trinucleotide repeat instability |
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| Authors: | Jinzhen Guo Luping Chen Guo-Min Li |
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| Institution: | 1.Department of Radiation Oncology,University of Texas Southwestern Medical Center,Dallas,USA;2.Department of Biochemistry and Molecular Medicine, Norris Comprehensive Cancer Center,University of Southern California Keck School of Medicine,Los Angeles,USA |
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| Abstract: | Trinucleotide repeat expansions cause over 30 severe neuromuscular and neurodegenerative disorders, including Huntington’s disease, myotonic dystrophy type 1, and fragile X syndrome. Although previous studies have substantially advanced the understanding of the disease biology, many key features remain unknown. DNA mismatch repair (MMR) plays a critical role in genome maintenance by removing DNA mismatches generated during DNA replication. However, MMR components, particularly mismatch recognition protein MutSβ and its interacting factors MutLα and MutLγ, have been implicated in trinucleotide repeat instability. In this review, we will discuss the roles of these key MMR proteins in promoting trinucleotide repeat instability. |
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