Martijn E T Dollé, Raoul V Kuiper, Marianne Roodbergen, Joke Robinson, Sisca de Vlugt, Susan W P Wijnhoven, Rudolf B Beems, Liset de la Fonteyne, Piet de With, Ingrid van der Pluijm, Laura J Niedernhofer, Paul Hasty, Jan Vijg, Jan H J Hoeijmakers, Harry van Steeg
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引用次数: 91
Abstract
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(-/Δ7), which is hemizygous for a single truncated Ercc1 allele, encoding a protein lacking the last seven amino acids. Ercc1(-/Δ7) 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(-/Δ7) 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(-/Δ7) mouse model.
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