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Zhou, Zhongjun; Liu, Baohua; Wang, Jianming; Chan, Kui Ming; Tjia, Wai Mui; Deng, Wen; Guan, Xinyuan; Huang, Jian-dong; Li, Kai Man; Chau, Pui Yin; Chen, David J; Pei, Duanqing; Pendas, Alberto M; Cadiñanos, Juan; López-Otín, Carlos; Tse, Hung Fat; Hutchison, Chris; Chen, Junjie; Cao, Yihai; Cheah, Kathryn S E; Tryggvason, Karl
Nature medicine, 07/2005, Letnik: 11, Številka: 7Journal Article
Premature aging syndromes often result from mutations in nuclear proteins involved in the maintenance of genomic integrity. Lamin A is a major component of the nuclear lamina and nuclear skeleton. Truncation in lamin A causes Hutchinson-Gilford progerial syndrome (HGPS), a severe form of early-onset premature aging. Lack of functional Zmpste24, a metalloproteinase responsible for the maturation of prelamin A, also results in progeroid phenotypes in mice and humans. We found that Zmpste24-deficient mouse embryonic fibroblasts (MEFs) show increased DNA damage and chromosome aberrations and are more sensitive to DNA-damaging agents. Bone marrow cells isolated from Zmpste24−/− mice show increased aneuploidy and the mice are more sensitive to DNA-damaging agents. Recruitment of p53 binding protein 1 (53BP1) and Rad51 to sites of DNA lesion is impaired in Zmpste24−/− MEFs and in HGPS fibroblasts, resulting in delayed checkpoint response and defective DNA repair. Wild-type MEFs ectopically expressing unprocessible prelamin A show similar defects in checkpoint response and DNA repair. Our results indicate that unprocessed prelamin A and truncated lamin A act dominant negatively to perturb DNA damage response and repair, resulting in genomic instability which might contribute to laminopathy-based premature aging.
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