Document Detail


From the rarest to the most common: insights from progeroid syndromes into skin cancer and aging.
MedLine Citation:
PMID:  19387478     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Brian C Capell; Brook E Tlougan; Seth J Orlow
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Publication Detail:
Type:  Journal Article; Review     Date:  2009-04-23
Journal Detail:
Title:  The Journal of investigative dermatology     Volume:  129     ISSN:  1523-1747     ISO Abbreviation:  J. Invest. Dermatol.     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-09-14     Completed Date:  2009-09-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0426720     Medline TA:  J Invest Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2340-50     Citation Subset:  IM    
Affiliation:
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York 10016, USA.
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MeSH Terms
Descriptor/Qualifier:
Aging, Premature / genetics*,  pathology
Cell Aging / genetics*
DNA Damage / genetics
Genomic Instability / genetics
Humans
Mutation / genetics
Progeria / genetics,  pathology
Skin Neoplasms / genetics*,  pathology
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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