Introduction of a Normal Human Chromosome 8 Corrects Abnormal Phenotypes of Werner Syndrome Cells Immortalized by Expressing an hTERT Gene

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Author(s)

    • Ariyoshi Kentaro ARIYOSHI Kentaro
    • Radiation Biology Laboratory, Radiation Research Center, Frontier Science Innovation Center, Organization for University-Industry-Government Cooperation, Osaka Prefecture University
    • Suzuki Keiji SUZUKI Keiji
    • Course of Life Sciences and Radiation Research, Graduate School of Biomedical Sciences, Nagasaki University
    • OSHIMURA Mitsuo
    • Division of Molecular Genetics and Biofunction, Department of Biomedical Science, Institute of Regenerative Medicine and Biofunction, Graduate School of Medical Sciences, Tottori University
    • ISHIZAKI Kanji
    • Central Laboratory and Radiation Biology, Research Institute, Aich Cancer Center
    • WATANABE Masami
    • Laboratory of Radiation Biology, Research Reactor Institute, Kyoto University
    • KODAMA Seiji
    • Radiation Biology Laboratory, Radiation Research Center, Frontier Science Innovation Center, Organization for University-Industry-Government Cooperation, Osaka Prefecture University

Abstract

Werner syndrome (WS) is an autosomal recessive disease characterized by premature aging and caused by mutations of the <i>WRN</i> gene mapped at 8p12. To examine functional complementation of WS phenotypes, we introduced a normal human chromosome 8 into a strain of WS fibroblasts (WS3RGB) immortalized by expressing a human telomerase reverse transcriptase subunit (h<i>TERT</i>) gene. Here, we demonstrate that the abnormal WS phenotypes including cellular sensitivities to 4-nitroquinoline-1-oxide (4NQO) and hydroxy urea (HU), and chromosomal radiosensitivity at G<sub>2</sub> phase are corrected by expression of the <i>WRN</i> gene mediated by introducing a chromosome 8. This indicates that those multiple abnormal WS phenotypes are derived from a primary, but not secondary, defect in the <i>WRN</i> gene.

Journal

  • Journal of Radiation Research

    Journal of Radiation Research 50(3), 253-259, 2009-05-16

    Journal of Radiation Research Editorial Committee

References:  28

Codes

  • NII Article ID (NAID)
    10025911840
  • NII NACSIS-CAT ID (NCID)
    AA00705792
  • Text Lang
    ENG
  • Article Type
    ART
  • ISSN
    04493060
  • NDL Article ID
    10230685
  • NDL Source Classification
    ZS45(科学技術--医学--放射線医学)
  • NDL Call No.
    Z53-P232
  • Data Source
    CJP  NDL  IR  J-STAGE 
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