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From the Rarest to the Most Common: Insights from Progeroid Syndromes into Skin Cancer and Aging
- Source :
- Journal of Investigative Dermatology. 129(10):2340-2350
- Publication Year :
- 2009
- Publisher :
- Elsevier BV, 2009.
-
Abstract
- Despite their rarity, diseases of premature aging, or “progeroid” syndromes, have provided important insights into basic mechanisms that may underlie cancer and normal aging. In this review, we highlight these recent developments in Hutchinson—Gilford progeria syndrome (HGPS), Werner syndrome, Bloom syndrome, Cockayne syndrome, trichothiodystrophy, ataxia-telangiectasia, Rothmund–Thomson syndrome, and xeroderma pigmentosum. Though they are caused by different mutations in various genes and often result in quite disparate phenotypes, deciphering the molecular bases of these conditions has served to highlight their underlying basic similarities. Studies of progeroid syndromes, particularly HGPS, the most dramatic form of premature aging, have contributed to our knowledge of fundamental processes of importance to skin biology, including DNA transcription, replication, and repair, genome instability, cellular senescence, and stem-cell differentiation.
- Subjects :
- Premature aging
congenital, hereditary, and neonatal diseases and abnormalities
Xeroderma pigmentosum
Skin Neoplasms
Trichothiodystrophy
Dermatology
Biology
Biochemistry
Cockayne syndrome
Progeroid syndromes
Genomic Instability
Progeria
medicine
Humans
Bloom syndrome
Molecular Biology
Cellular Senescence
Werner syndrome
Genetics
integumentary system
nutritional and metabolic diseases
Aging, Premature
Syndrome
Cell Biology
medicine.disease
Mutation
DNA Damage
Subjects
Details
- ISSN :
- 0022202X
- Volume :
- 129
- Issue :
- 10
- Database :
- OpenAIRE
- Journal :
- Journal of Investigative Dermatology
- Accession number :
- edsair.doi.dedup.....8122228730a15a220dfec86b2dc5c533
- Full Text :
- https://doi.org/10.1038/jid.2009.103