| http://purl.uniprot.org/citations/15980864 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/15980864 | http://www.w3.org/2000/01/rdf-schema#comment | "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 embryonic fibroblasts (MEFs) show increased DNA damage and chromosome aberrations and are more sensitive to DNA-damaging agents. Bone marrow cells isolated from Zmpste24-/-mice show increased aneuploidy and the mice are more sensitive to DNA-damaging agents. Recruitment of p53 binding protein 1 (53BP1) and Rad51 to sites of DNA lesion is impaired in Zmpste24-/-MEFs and in HGPS fibroblasts, resulting in delayed checkpoint response and defective DNA repair. Wild-type MEFs ectopically expressing unprocessible prelamin A show similar defects in checkpoint response and DNA repair. Our results indicate that unprocessed prelamin A and truncated lamin A act dominant negatively to perturb DNA damage response and repair, resulting in genomic instability which might contribute to laminopathy-based premature aging."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.org/dc/terms/identifier | "doi:10.1038/nm1266"xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Chen J."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Deng W."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Cao Y."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Liu B."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Lopez-Otin C."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Pei D."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Wang J."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Zhou Z."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Guan X."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Chan K.M."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Tryggvason K."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Chen D.J."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Cheah K.S."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Cadinanos J."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Pendas A.M."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Huang J.D."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Tse H.F."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Li K.M."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Chau P.Y."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Hutchison C."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/author | "Tjia W.M."xsd:string |
| http://purl.uniprot.org/citations/15980864 | http://purl.uniprot.org/core/date | "2005"xsd:gYear |