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PMID: 18006573 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Review

Human premature aging, DNA repair and RecQ helicases.

Nucleic acids research ·Vol. 35 ·No. 22 ·2007-00-00 ·Pages 7527-44

Brosh RM, Bohr VA

Abstract

Genomic instability leads to mutations, cellular dysfunction and aberrant phenotypes at the tissue and organism levels. A number of mechanisms have evolved to cope with endogenous or exogenous stress to prevent chromosomal instability and maintain cellular homeostasis. DNA helicases play important roles in the DNA damage response. The RecQ family of DNA helicases is of particular interest since several human RecQ helicases are defective in diseases associated with premature aging and cancer. In this review, we will provide an update on our understanding of the specific roles of human RecQ helicases in the maintenance of genomic stability through their catalytic activities and protein interactions in various pathways of cellular nucleic acid metabolism with an emphasis on DNA replication and repair. We will also discuss the clinical features of the premature aging disorders associated with RecQ helicase deficiencies and how they relate to the molecular defects.

MeSH Terms
Aging, Premature/diagnosis,genetics Chromosomal Instability DNA Repair DNA Replication Exodeoxyribonucleases Humans Mutation Neoplasms/drug therapy,genetics RecQ Helicases/antagonists & inhibitors,genetics,physiology Replication Protein A/metabolism Sister Chromatid Exchange Telomere/metabolism Werner Syndrome Helicase
Chemicals
Replication Protein A Exodeoxyribonucleases RecQ Helicases WRN protein, human Werner Syndrome Helicase
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Brosh Robert M
Laboratory of Molecular Gerontology, National Institute on Aging, NIH, 5600 Nathan Shock Drive, Baltimore, MD 21224, USA.
Bohr Vilhelm A
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Article Info
Journal
Nucleic acids research
Abbr.
Nucleic Acids Res
ISSN
1362-4962
DOI
10.1093/nar/gkm1008
Published
2007-00-00
Epub
2007-00-15
Pages
7527-44
Language
English
Region
England
NLM ID
0411011
PMCID
PMC2190726
Subset
IM
External Links
PubMed source DOI: 10.1093/nar/gkm1008 PMC Full Text View journal details
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