Article
Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation.
Nature - 22 Sept 2005
Varela Ignacio, Cadiñanos Juan, Pendás Alberto M, Gutiérrez-Fernández Ana, Folgueras Alicia R, Sánchez Luis M, Zhou Zhongjun, Rodríguez Francisco J, Stewart Colin L, Vega José A, Tryggvason Karl, Freije José M P, López-Otín Carlos
Abstract excerpt
Zmpste24 (also called FACE-1) is a metalloproteinase involved in the maturation of lamin A (Lmna), an essential component of the nuclear envelope. Both Zmpste24- and Lmna-deficient mice exhibit profound nuclear architecture abnormalities and multiple histopathological defects that phenocopy an accelerated ageing process. Similarly, diverse human progeroid syndromes are caused by mutations in ZMPSTE24 or LMNA...
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