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Review
. 2007;35(22):7527-44.
doi: 10.1093/nar/gkm1008. Epub 2007 Nov 15.

Human premature aging, DNA repair and RecQ helicases

Robert M Brosh Jr  1 , Vilhelm A Bohr
Affiliations
Review

Human premature aging, DNA repair and RecQ helicases

Robert M Brosh Jr et al. Nucleic Acids Res. 2007.

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.

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Figures

Figure 1.
Figure 1.
DNA repair pathways responsible for the correction of endogenous or exogenously induced DNA damage.
Figure 2.
Figure 2.
Involvement of RecQ helicases at replication forks and in DNA repair pathways to maintain genomic stability. See text for details.
Figure 3.
Figure 3.
Molecular steps and proteins of base excision repair pathways.
See this image and copyright information in PMC

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Publication types