RecQ helicases: caretakers of the genome
Top Cited Papers
- 1 March 2003
- journal article
- review article
- Published by Springer Nature in Nature Reviews Cancer
- Vol. 3 (3) , 169-178
- https://doi.org/10.1038/nrc1012
Abstract
RecQ helicases represent a highly conserved family that is required for the maintenance of genome integrity. In humans, defects in any of three RecQ family members (BLM, WRN or RECQ4) give rise to cancer predisposition disorders. These are Bloom's, Werner's and Rothmund–Thomson syndromes, respectively. RecQ helicases are considered to be 'caretaker' tumour suppressors that suppress neoplastic transformation through control of chromosomal stability. Many other similar caretakers are functionally linked to the RecQ helicases, indicating a possible common molecular basis for tumorigenesis in several apparently distinct cancer predisposition disorders. Human RecQ helicases make multiple physical interactions with other nuclear proteins that are required for DNA metabolism. Many of these interactions have a functional effect on the activity of one or both partners. RecQ helicases are proposed to function at the interface between DNA replication and recombination to 'repair' damaged replication forks.Keywords
This publication has 109 references indexed in Scilit:
- Functional Link between BLM Defective in Bloom's Syndrome and the Ataxia-telangiectasia-mutated Protein, ATMJournal of Biological Chemistry, 2002
- p53 Modulates the Exonuclease Activity of Werner Syndrome ProteinPublished by Elsevier ,2001
- The Bloom's Syndrome Protein (BLM) Interacts with MLH1 but Is Not Required for DNA Mismatch RepairJournal of Biological Chemistry, 2001
- The Werner syndrome protein: an updateBioEssays, 2000
- Functional Interaction between Ku and the Werner Syndrome Protein in DNA End ProcessingJournal of Biological Chemistry, 2000
- DNA Helicases, Genomic Instability, and Human Genetic DiseaseAnnual Review of Genomics and Human Genetics, 2000
- Werner Syndrome ProteinJournal of Biological Chemistry, 1998
- Stage-specific apoptosis, developmental delay, and embryonic lethality in mice homozygous for a targeted disruption in the murine Bloom’s syndrome geneGenes & Development, 1998
- Telomeres shorten during ageing of human fibroblastsNature, 1990
- Mutation and Cancer: Statistical Study of RetinoblastomaProceedings of the National Academy of Sciences, 1971