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Lamin C protein deficiency in the primary fibroblasts from a new laminopathy case with ovarian cystadenoma

Chinese Medical Journal 2010年第16期123卷 2237-2243页

作者： CAI Meng-yin LIANG Hua LI Ming BI Yan CHEN Xiang SUN Wei-ping WENG Jian-ping Department of Endocrinology Third Affiliated Hospital of Sun Yat-sen University Guangzhou Guangdong 510630 China

Background Laminopathies are a group of rare genetic disorders characterized by multiple-tissue *** describe a new laminopathy with ovarian cystadenoma and explore its molecular *** The case is a 15-year-old girl who presents the prominent progeroid disorders, multiple system degeneration and early-onset cystadenoma of the *** genes including LMNA, ZMPSTE24, PPAR G, INSR and WRN were sequenced to screen for DNA *** mRNA and protein expression levels of LMNA were examined in primary *** pathophysiological events such as morphologic alterations, cell senescence, cell proliferation,apoptosis and pRb as well as p53 protein expressions were also investigated in primary *** No mutation was identified in the candidate genes *** abnormalities including nuclear blebs,mislocalization of lamin A/C were evident in the patient ***, nucleus exhibited nuclear herniation and almost complete loss of peripheral *** addition, lamin C protein expression was markedly reduced whereas lamin A protein level was normal and no prelamin A was detected in the primary *** the senescence-associated β-galactosidase staining of patient＇ cells was negative, cells in S phase increased in accompany with a decrease in pRb protein ***, increases in apoptotic cell death and p53 expression were *** Our data suggest that selective deficiency of lamin C protein is associated with a case of laminopathy with ovarian *** abnormalities in nuclear structure and alterations in gene expression such as the decrease in pRb and increase in p53 may be responsible for the multiple tissue degeneration.

关键词： laminopathy lamin C premature aging pRb p53

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Genomice Instability in laminopathy-based Premature Ageing

Genomice Instability in Laminopathy-based Premature Ageing

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中国细胞生物学学会第九次会员代表大会暨青年学术大会

作者： Bao-Hua Liu Zhong-Jun Zhou (Department of Biochemistry,University of Hong Kong,Hong Kong,China)

Through genetic approach,we have identified the in vivo substrate for Zmpste24,a metalloproteinase on ER. Zmpste24 processes prelamin A to its mature *** of Zmpste24 results in accumulation of unprocessed prelamin A a... 详细信息

关键词： genomic instability DNA repair laminopathy premature aging

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