ERCC5
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DNA repair protein complementing XP-G cells is a protein that in humans is encoded by the ERCC5 gene.
DNA repair protein complementing XP-G cells is a protein that in humans is encoded by the ERCC5 gene.
Syndromes
Mutational defects in the Ercc5(Xpg) gene can cause either the cancer-prone condition xeroderma pigmentosum (XP) alone, or in combination with the severe neurodevelopmental disorder Cockayne syndrome (CS) or the infantile lethal cerebro-oculo-facio-skeletal syndrome.
Mouse model
An Ercc5(Xpg) mutant mouse model presented features of premature aging including cachexia and osteoporosis with pronounced degenerative phenotypes in both liver and brain. These mutant mice developed a multi-system premature aging degenerative phenotype that appears to strengthen the link between DNA damage and aging. (see DNA damage theory of aging). Dietary restriction, which extends lifespan of wild-type mice, also substantially increased the lifespan of Ercc5(Xpg) mutant mice. Dietary restriction of the mutant mice, while delaying aging, also appeared to slow the accumulation of genome wide DNA damage and to preserve transcriptional output, thus contributing to improved cell viability.
External links
Text derived from https://en.wikipedia.org/w/index.php?oldid=1316285946 under CC BY-SA 4.0; modifications have been made.