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Rising from the RecQ-age: the role of human RecQ helicases in genome maintenance Bohr VATrends Biochem Sci 2008[Dec]; 33 (12): 609-20The RecQ helicases are guardians of the genome. Members of this conserved family of proteins have a key role in protecting and stabilizing the genome against deleterious changes. Deficiencies in RecQ helicases can lead to high levels of genomic instability and, in humans, to premature aging and increased susceptibility to cancer. Their diverse roles in DNA metabolism, which include a role in telomere maintenance, reflect interactions with multiple cellular proteins, some of which are multifunctional and also have very diverse functions. The results of in vitro cellular and biochemical studies have been complimented by recent in vivo studies using genetically modified mouse strains. Together, these approaches are helping to unravel the mechanism(s) of action and biological functions of the RecQ helicases.|*Genome, Human[MESH]|Aging, Premature/genetics[MESH]|Animals[MESH]|Bloom Syndrome/genetics[MESH]|DNA Breaks, Double-Stranded[MESH]|DNA Repair/physiology[MESH]|DNA Replication/physiology[MESH]|Disease Models, Animal[MESH]|Exodeoxyribonucleases/physiology[MESH]|Humans[MESH]|Mice[MESH]|Protein Processing, Post-Translational[MESH]|RecQ Helicases/*physiology[MESH]|Recombination, Genetic[MESH]|Rothmund-Thomson Syndrome/genetics[MESH]|Telomere/physiology[MESH]|Transcription, Genetic/physiology[MESH]|Werner Syndrome Helicase[MESH]|Werner Syndrome/genetics[MESH] |
