Adaptive stress response in segmental progeria resembles long-lived dwarfism and calorie restriction in mice.

ABSTRACT: How congenital defects causing genome instability can result in the pleiotropic symptoms reminiscent of aging but in a segmental and accelerated fashion remains largely unknown. Most segmental progerias are associated with accelerated fibroblast senescence, suggesting that cellular senescence is a likely contributing mechanism. Contrary to expectations, neither accelerated senescence nor acute oxidative stress hypersensitivity was detected in primary fibroblast or erythroblast cultures from multiple progeroid mouse models for defects in the nucleotide excision DNA repair pathway, which share premature aging features including postnatal growth retardation, cerebellar ataxia, and death before weaning. Instead, we report a prominent phenotypic overlap with long-lived dwarfism and calorie rest

SUBMITTER: van de Ven M 

PROVIDER: S-EPMC1698946 | biostudies-literature | 2006 Dec

REPOSITORIES: biostudies-literature