Category Archives: progeroid syndrome

introduction of a normal human chromosome 8 corrects Werner Syndrome cells

Kentaro Ariyoshi and colleagues successfully introduced a normal human chromosome 8 into Werner Syndrome (WS) cells(fibroblasts) immortalized by expressing a human telomere reverse transcriptase subunit (hTERT) gene. In their study (published in the Journal of Radiation Research), they demonstrated that the abnormal WS cellular phenotypes like sensitivity to 4-nitroquinoline-1-oxide (4NQO) and hydroxyurea (HU), and chromosomal radiosensitivity at G(2) phase can be corrected by expression of the WRN gene upon introduction of a chromosome 8 via microcell fusion. Their results provide more evidence that the multiple abnormal WS phenotypes (clinical, cellular, and chromosomal) are derived from a primary, but not secondary, defect in the WRN gene.

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Filed under progeria, progeroid syndrome, Werner Syndrome, Werners Syndrome, WRN, WS