%0 Journal Article
%T Broad segmental progeroid changes in short-lived Ercc1  /¦¤7 mice
%A Martijn E.T. Doll¨¦
%A Raoul V. Kuiper
%A Marianne Roodbergen
%A Joke Robinson
%J Pathobiology of Aging & Age-related Diseases
%D 2011
%I 
%R 10.3402/pba.v1i0.7219
%X 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  /¦¤7, which is hemizygous for a single truncated Ercc1 allele, encoding a protein lacking the last seven amino acids. Ercc1  /¦¤7 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  /¦¤7 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  /¦¤7 mouse model.
%K Ercc1
%K mouse
%K aging
%K life span
%K cross sectional
%K pathology
%K immunosenescense
%K body weight
%K organ weight
%K FVB
%K C57BL/6
%K genome maintenance
%U http://www.pathobiologyofaging.net/index.php/pba/article/view/7219/pdf_151