| First Author | Dollé ME | Year | 2011 |
| Journal | Pathobiol Aging Age Relat Dis | Volume | 1 |
| PubMed ID | 22953029 | Mgi Jnum | J:202585 |
| Mgi Id | MGI:5520042 | Doi | 10.3402/pba.v1i0.7219 |
| Citation | Dolle ME, et al. (2011) Broad segmental progeroid changes in short-lived Ercc1(-/Delta7) mice. Pathobiol Aging Age Relat Dis 1 |
| abstractText | Genome maintenance is considered a prime longevity assurance mechanism as apparent from many progeroid human syndromes that are caused by genome maintenance defects. The ERCC1 protein is involved in three genome maintenance systems: nucleotide excision repair, interstrand cross-link repair, and homologous recombination. Here we describe in-life and post-mortem observations for a hypomorphic Ercc1 variant, Ercc1(-/Delta7), which is hemizygous for a single truncated Ercc1 allele, encoding a protein lacking the last seven amino acids. Ercc1(-/Delta7) mice were much smaller and median life span was markedly reduced compared to wild-type siblings: 20 and 118 weeks, respectively. Multiple signs and symptoms of aging were found to occur at an accelerated rate in the Ercc1(-/Delta7) mice as compared to wild-type controls, including a decline in weight of both whole body and various organs, numerous histopathological lesions, and immune parameters. Together they define a segmental progeroid phenotype of the Ercc1(-/Delta7) mouse model. |
