Mutant huntingtin impairs Ku70-mediated DNA repair
نویسندگان
چکیده
منابع مشابه
Mutant huntingtin impairs Ku70-mediated DNA repair
DNA repair defends against naturally occurring or disease-associated DNA damage during the long lifespan of neurons and is implicated in polyglutamine disease pathology. In this study, we report that mutant huntingtin (Htt) expression in neurons causes double-strand breaks (DSBs) of genomic DNA, and Htt further promotes DSBs by impairing DNA repair. We identify Ku70, a component of the DNA dama...
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DNA damage accumulates in genome DNA during the long life of neurons, thus DNA damage repair is indispensable to keep normal functions of neurons. We previously reported that Ku70, a critical molecule for DNA double strand break (DSB) repair, is involved in the pathology of Huntington's disease (HD). Mutant huntingtin (Htt) impaired Ku70 function via direct interaction, and Ku70 supplementation...
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ژورنال
عنوان ژورنال: The Journal of Experimental Medicine
سال: 2010
ISSN: 0022-1007,1540-9538
DOI: 10.1084/jem2075oia16