Journal
NUCLEUS
Volume 9, Issue 1, Pages 368-379Publisher
TAYLOR & FRANCIS INC
DOI: 10.1080/19491034.2018.1476793
Keywords
Lamin; DNA replication; progeria; nuclear lamina; aging; DNA damage
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Funding
- Fondazione Telethon [GEP15033]
- MIUR Ateneo
- Progeria Research Foundation [PRF 2016-67]
- EMBO short-term fellowship 2017
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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 polo, 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.
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