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- W2809546154 abstract "Progeroid syndromes induced by mutations in lamin A or in its interactors - named progeroid laminopathies - are model systems for the dissection of the molecular pathways causing physiological and premature aging. A large amount of data, based mainly on the Hutchinson Gilford Progeria syndrome (HGPS), one of the best characterized progeroid laminopathy, has highlighted the role of lamins in multiple DNA activities, including replication, repair, chromatin organization and telomere function. On the other hand, the phenotypes generated by mutations affecting genes directly acting on DNA function, as mutations in the helicases WRN and BLM or in the polymerase polδ, share many of the traits of progeroid laminopathies. These evidences support the hypothesis of a concerted implication of DNA function and lamins in aging. We focus here on these aspects to contribute to the comprehension of the driving forces acting in progeroid syndromes and premature aging." @default.
- W2809546154 created "2018-06-29" @default.
- W2809546154 creator A5007790566 @default.
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- W2809546154 creator A5034284174 @default.
- W2809546154 creator A5079371643 @default.
- W2809546154 creator A5088657110 @default.
- W2809546154 date "2018-06-23" @default.
- W2809546154 modified "2023-10-12" @default.
- W2809546154 title "Genomic instability and DNA replication defects in progeroid syndromes" @default.
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