Journal
CURRENT BIOLOGY
Volume 11, Issue 12, Pages 962-966Publisher
CELL PRESS
DOI: 10.1016/S0960-9822(01)00267-6
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Funding
- NCI NIH HHS [CA54326, CA79658] Funding Source: Medline
- NIA NIH HHS [K08 AG001019] Funding Source: Medline
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Nijmegen breakage syndrome (NBS) is a rare human disease displaying chromosome instability, radiosensitivity, cancer predisposition, immunodeficiency, and other defects [1, 2]. NBS is complexed with MRE11 and RAD50 in a DNA repair complex [3-5] and is localized to telomere ends in association with TRF proteins [6, 7]. We show that blood cells from NBS patients have shortened telomere DNA ends. Likewise, cultured NBS fibroblasts that exhibit a premature growth cessation were observed with correspondingly shortened telomeres. Introduction of the catalytic subunit of telomerase, TERT, was alone sufficient to increase the proliferative capacity of NBS fibroblasts. However, Has, but not TERT, restores the capacity of NBS cells to survive gamma irradiation damage. Strikingly, NBS promotes telomere elongation in conjunction with TERT in NBS fibroblasts. These results suggest that NBS is a required accessory protein for telomere extension. Since NBS patients have shortened telomeres, these defects may contribute to the chromosome instability and disease associated with NBS patients. (C) 2001 Elsevier Science Ltd. All rights reserved.
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