p53-mediated apoptosis is attenuated in Werner syndrome cells
Article Abstract:
Apoptosis which has been mediated by p53 is attenuated in Werner syndrome (WS) cells. WRN is a gene that shows mutations in those who have WS, a premature-aging/cancer-susceptibility syndrome. The WRN DNA helicase belongs to the DExH-containing DNA helicase superfamily that includes BLM, XPB, and XPD. p53 binds to the WRN protein in vivo and in vitro through the carboxyl terminus. WS fibroblasts have a p53-mediated apoptotic response that is attenuated. The deficiency can be gotten rid of by expression of wild-type WRN. It appears that p53 can perhaps induce apoptosis through modulation of specific DExH-containing DNA helicases. Implications for the cancer predisposition seen in WS patients may exist.
Publication Name: Genes & Development
Subject: Biological sciences
ISSN: 0890-9369
Year: 1999
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Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1[alpha]/LRP130 complex
Article Abstract:
A study was conducted to investigate defects in energy homeostasis in Leigh syndrome French Canadian (LSFC) variant through PGC-1[alpha]/LRP130 complex. A complex of proteins assembled by PGC-1[alpha] from mammalian cell extracts was purified and it was shown that LRP130 constitutes a prominent component of this complex and with PGC-1[alpha] linked to defective hepatic energy homeostasis in LSFC and reveals a novel regulatory mechanism of glucose homeostasis.
Publication Name: Genes & Development
Subject: Biological sciences
ISSN: 0890-9369
Year: 2006
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Ku complex interacts with and stimulates the Werner protein
Article Abstract:
Werner syndrome (WS, a premature aging disorder) and the biochemical aspects of the cells of WS patients have been studied. Ku complex was found to interact with and stimulate the Werner protein. Ku70 and Ku86 have been found to be proteins that interact with WRNp. Ku proteins did not affect ATPase or helicase activity, but strongly stimulated specific exonuclease activity.
Publication Name: Genes & Development
Subject: Biological sciences
ISSN: 0890-9369
Year: 2000
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