Human werner syndrome DNA helicase unwinds tetrahelical structures of the fragile X syndrome repeat sequence d(CGG)n - PubMed (original) (raw)
. 1999 Apr 30;274(18):12797-802.
doi: 10.1074/jbc.274.18.12797.
Affiliations
- PMID: 10212265
- DOI: 10.1074/jbc.274.18.12797
Free article
Human werner syndrome DNA helicase unwinds tetrahelical structures of the fragile X syndrome repeat sequence d(CGG)n
M Fry et al. J Biol Chem. 1999.
Free article
Abstract
Formation of hairpin and tetrahelical structures by a d(CGG) trinucleotide repeat sequence is thought to cause expansion of this sequence and to engender fragile X syndrome. Here we show that human Werner syndrome DNA helicase (WRN), a member of the RecQ family of helicases, efficiently unwinds G'2 bimolecular tetraplex structures of d(CGG)7. Unwinding of d(CGG)7 by WRN requires hydrolyzable ATP and Mg2+ and is proportional to the amount of added helicase and to the time of incubation. The efficiencies of unwinding of G'2 d(CGG)7 tetraplex with 7 nucleotide-long single-stranded tails at their 3' or 5' ends are, respectively, 3.5- and 2-fold greater than that of double-stranded DNA. By contrast, WRN is unable to unwind a blunt-ended d(CGG)7 tetraplex, bimolecular tetraplex structures of a telomeric sequence 5'-d(TAGACATG(TTAGGG)2TTA)-3', or tetramolecular quadruplex forms of an IgG switch region sequence 5'-d(TACAGGGGAGCTGGGGTAGA)-3'. The ability of WRN to selectively unwind specific tetrahelices may reflect a specific role of this helicase in DNA metabolism.
Similar articles
- Interactions between the Werner syndrome helicase and DNA polymerase delta specifically facilitate copying of tetraplex and hairpin structures of the d(CGG)n trinucleotide repeat sequence.
Kamath-Loeb AS, Loeb LA, Johansson E, Burgers PM, Fry M. Kamath-Loeb AS, et al. J Biol Chem. 2001 May 11;276(19):16439-46. doi: 10.1074/jbc.M100253200. Epub 2001 Feb 8. J Biol Chem. 2001. PMID: 11279038 - Interruption of the fragile X syndrome expanded sequence d(CGG)(n) by interspersed d(AGG) trinucleotides diminishes the formation and stability of d(CGG)(n) tetrahelical structures.
Weisman-Shomer P, Cohen E, Fry M. Weisman-Shomer P, et al. Nucleic Acids Res. 2000 Apr 1;28(7):1535-41. doi: 10.1093/nar/28.7.1535. Nucleic Acids Res. 2000. PMID: 10710419 Free PMC article. - Unwinding the molecular basis of the Werner syndrome.
Shen J, Loeb LA. Shen J, et al. Mech Ageing Dev. 2001 Jul 15;122(9):921-44. doi: 10.1016/s0047-6374(01)00248-2. Mech Ageing Dev. 2001. PMID: 11348659 Review. - The Werner syndrome helicase-nuclease--one protein, many mysteries.
Fry M. Fry M. Sci Aging Knowledge Environ. 2002 Apr 3;2002(13):re2. doi: 10.1126/sageke.2002.13.re2. Sci Aging Knowledge Environ. 2002. PMID: 14602980 Review.
Cited by
- G-quadruplexes originating from evolutionary conserved L1 elements interfere with neuronal gene expression in Alzheimer's disease.
Hanna R, Flamier A, Barabino A, Bernier G. Hanna R, et al. Nat Commun. 2021 Mar 23;12(1):1828. doi: 10.1038/s41467-021-22129-9. Nat Commun. 2021. PMID: 33758195 Free PMC article. - The yeast Sgs1 helicase is differentially required for genomic and ribosomal DNA replication.
Versini G, Comet I, Wu M, Hoopes L, Schwob E, Pasero P. Versini G, et al. EMBO J. 2003 Apr 15;22(8):1939-49. doi: 10.1093/emboj/cdg180. EMBO J. 2003. PMID: 12682026 Free PMC article. - Interaction of human DNA topoisomerase I with G-quartet structures.
Arimondo PB, Riou JF, Mergny JL, Tazi J, Sun JS, Garestier T, Hélène C. Arimondo PB, et al. Nucleic Acids Res. 2000 Dec 15;28(24):4832-8. doi: 10.1093/nar/28.24.4832. Nucleic Acids Res. 2000. PMID: 11121473 Free PMC article. - Analysis of the unwinding activity of the dimeric RECQ1 helicase in the presence of human replication protein A.
Cui S, Arosio D, Doherty KM, Brosh RM Jr, Falaschi A, Vindigni A. Cui S, et al. Nucleic Acids Res. 2004 Apr 19;32(7):2158-70. doi: 10.1093/nar/gkh540. Print 2004. Nucleic Acids Res. 2004. PMID: 15096578 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical