文章基本信息

标题：Introduction of a Normal Human Chromosome 8 Corrects Abnormal Phenotypes of Werner Syndrome Cells Immortalized by Expressing an hTERT Gene
其他标题：Introduction of a Normal Human Chromosome 8 Corrects Abnormal Phenotypes of Werner Syndrome Cells Immortalized by Expressing an hTERT Gene
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作者：Kentaro ARIYOSHI ; Keiji SUZUKI ; Makoto GOTO 等
期刊名称：Journal of Radiation Research
印刷版ISSN：0449-3060
电子版ISSN：1349-9157
出版年度：2009
卷号：50
期号：3
页码：253-259
DOI：10.1269/jrr.08111
摘要：Werner syndrome (WS) is an autosomal recessive disease characterized by premature aging and caused by mutations of the WRN 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 TERT ) 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 2 phase are corrected by expression of the WRN 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 WRN gene.
关键词：Werner syndrome; Chromosome transfer; Functional complementation