2005
DOI: 10.1038/nm1266
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Abstract: 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 embryo… Show more

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Cited by 564 publications
(569 citation statements)
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“…These alterations in nuclear architecture induced by prelamin A and progerin have also been linked to a defective recruitment of DNA repair factors and increased genomic instability (Liu et al 2005). In agreement with these findings, transcriptional profiling of tissues from Zmpste24-deficient mice revealed a clear up-regulation of p53 target genes, which becomes more pronounced as the progeroid phenotype of these animals becomes more dramatic .…”
Section: Zmpste24-deficient Mice As a Model Of Accelerated Ageingsupporting
confidence: 56%
“…These alterations in nuclear architecture induced by prelamin A and progerin have also been linked to a defective recruitment of DNA repair factors and increased genomic instability (Liu et al 2005). In agreement with these findings, transcriptional profiling of tissues from Zmpste24-deficient mice revealed a clear up-regulation of p53 target genes, which becomes more pronounced as the progeroid phenotype of these animals becomes more dramatic .…”
Section: Zmpste24-deficient Mice As a Model Of Accelerated Ageingsupporting
confidence: 56%
“…That study, as well as another study [74], suggested that the loss of ZMPSTE24 results in increased DNA damage and activation of a cell senescence program, and that this could be partly ameliorated by deleting p53 and by reducing synthesis of farnesyl-prelamin A.…”
Section: Zmpste24-deficient Micementioning
confidence: 82%
“…Zmpste24 deficient mice (Zmpste24 −/− ) were created by two groups [68,69,74]. Intriguingly, mice with complete loss of Zmpste24 do not develop the severe disease in human patients with restrictive dermopathy that is associated with perinatal death [73].…”
Section: Zmpste24-deficient Micementioning
confidence: 99%
“…The cellular defects found in progeroid diseases that also characterize normal human aging include DNA damage and genome instability, telomere attrition, epigenetic alterations of histones, aberrations in the nuclear lamina, and cell senescence (de Boer et al, 2002; Liu et al., 2005; Osorio, Ugalde, et al., 2011; Varela et al., 2005). …”
Section: Discussionmentioning
confidence: 99%