期刊
CELL CYCLE
卷 10, 期 14, 页码 2269-2275出版社
TAYLOR & FRANCIS INC
DOI: 10.4161/cc.10.14.16495
关键词
DNA repair; nervous system; neurodegeneration; DNA ligase III; DNA damage; XRCC1; mitochondria; mtDNA
类别
资金
- National Institutes of Health [NS-37956, CA-21765]
- Cancer Center [P30 CA21765]
- American Lebanese and Syrian Associated Charities of St. Jude Children's Research Hospital
DNA strand break repair is essential for the prevention of multiple human diseases, particularly those which feature neuropathology. To further understand the pathogenesis of these syndromes we recently developed animal models in which the DNA single-strand break repair (SSBR) components, XRCC1 and DNA Ligase III (LIG3), were inactivated in the developing nervous system. Although biochemical evidence suggests that inactivation of XRCC1 and LIG3 should share common biological defects, we found profound phenotypic differences between these two models, implying distinct biological roles for XRCC1 and LIG3 during DNA repair. Rather than a key role in nuclear DNA repair, we found LIG3 function was central to mitochondrial DNA maintenance. Instead, our data indicate that DNA Ligase 1 is the main DNA ligase for XRCC1-mediated DNA repair. These studies refine our understanding of DNA SSBR and the etiology of neurological disease.
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