Genomic instability in laminopathy-based premature aging

  title={Genomic instability in laminopathy-based premature aging},
  author={Baohua Liu and J. Wang and K. Chan and Wai Mui Tjia and W. Deng and Xin-Yuan Guan and J. Huang and K. Li and Pui Yin Chau and D. Chen and D. Pei and A. Pend{\'a}s and J. Cadi{\~n}anos and C. L{\'o}pez-Ot{\'i}n and H. Tse and C. Hutchison and J. Chen and Y. Cao and Kathryn S. E. Cheah and K. Tryggvason and Zhongjun Zhou},
  journal={Nature Medicine},
  • Baohua Liu, J. Wang, +18 authors Zhongjun Zhou
  • Published 2005
  • Biology, Medicine
  • Nature Medicine
  • 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… CONTINUE READING
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