DNA mismatch repair in trinucleotide repeat instability

被引：0
|
作者：
Jinzhen Guo
Luping Chen
Guo-Min Li
机构：
[1] University of Texas Southwestern Medical Center,Department of Radiation Oncology
[2] University of Southern California Keck School of Medicine,Department of Biochemistry and Molecular Medicine, Norris Comprehensive Cancer Center
来源：
Science China Life Sciences | 2017年 / 60卷
关键词：
DNA mismatch repair; trinucleotide repeat instability; neurodegenerative diseases; MutSβ;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
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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页码：1087 / 1092
页数：5
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