Reports from National Institutes of Health highlight recent research in Cockayne syndrome
2007 OCT 22 -- "'The Cockayne syndrome B (CSB) protein-defective in a majority of patients suffering from the rare autosomal disorder CS-is a member of the SWI2/SNF2 family with roles in DNA repair and transcription. We demonstrate herein that purified recombinant CSB and the major human apurinic/apyrimidinic (AP) endonuclease, APE1, physically and functionally interact," investigators in the United States report. "CSB stimulates the AP site incision activity of APE1 on normal (i.e. fully paired) and bubble AP-DNA substrates, with the latter being more pronounced ( =up to 6-fold). This activation is ATP-independent, and specific for the human CSB and full-length APE1 protein, as no CSB-dependent stimulation was observed with Escherichia coli endonuclease IV or an N-terminal truncated APE1 fragment. CSB and APE1 were also found in a common protein complex in human cell extracts, and recombinant CSB, when added back to CSB-deficient whole cell extracts, resulted in increased total AP site incision capacity," wrote H.K. Wong and colleagues, National Institutes of Health. The researchers concluded: "Moreover, human fibroblasts defective in CSB were found to be hypersensitive to both methyl methanesulfonate (MMS) and 5-hydroxymethyl 20-deoxyuridine, agents that introduce base excision repair (BER) DNA substrates/intermediates." Wong and colleagues published their study in Nucleic Acids Research (Cockayne syndrome B protein stimulates apurinic endonuclease 1 activity and protects against agents that introduce base excision repair intermediates. Nucleic Acids Research, 2007;35(12):4103-4113). For additional information, contact D.M. Wilson, NIA, Laboratory Molecular Gerontology, National Institutes of Health, 5600 Nathan Shock Dr., Baltimore, MD 21224, USA. The publisher of the journal Nucleic Acids Research can be contacted at: Oxford University Press, Great Clarendon St., Oxford OX2 6DP, England. Keywords: United States, Baltimore, Cockayne Syndrome, DNA Repair, DNA Research, Deoxyribonucleic Acid, Endonuclease, Enzyme Research, Enzymology, Neurology, Polymerase, Proteomics, National Institutes of Health. This article was prepared by Pain & Central Nervous System Week editors from staff and other reports. Copyright 2007, Pain & Central Nervous System Week via NewsRx.com.
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