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Telomere maintenanc...
Telomere maintenance and cell cycle regulation in spontaneously immortalized T-cell lines from Nijmegen breakage syndrome patients
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- Siwicki, Jan Konrad (författare)
- Department of Immunology, M. Skladowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland
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- Degerman, Sofie, 1977- (författare)
- Umeå universitet,Patologi
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- Chrzanowska, Krystyna H (författare)
- Department of Medical Genetics, Childrens Memorial Health Institute, Warsaw, Poland
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- Roos, Göran (författare)
- Umeå universitet,Patologi
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Department of Immunology, M Skladowska-Curie Memorial Cancer Center and Institute of Oncology, Warsaw, Poland Patologi (creator_code:org_t)
- 2003
- 2003
- Engelska.
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Ingår i: Experimental Cell Research. - 0014-4827 .- 1090-2422. ; 287:1, s. 178-189
- Relaterad länk:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- Nijmegen breakage syndrome (NBS) is a rare genetic instability syndrome associated with a high incidence of lymphoid malignancies. The NBS1 protein has been implicated in telomere biology suggesting that cells from NBS patients might have deficient telomere maintenance capacity. In this study we characterized spontaneously immortalized T-cell lines derived from three NBS patients regarding growth characteristics, telomere biology, expression of cell-cycle regulators, and response to DNA damage to understand the role of NBS1 in the immortalization process. In all the NBS T-cell lines the acquisition of an immortal phenotype was associated with telomere length stabilization, high telomerase activity, and increased mRNA expression of the catalytic subunit of telomerase (hTERT), together with c-myc up-regulation. Our findings provide evidence that telomere length maintenance was intact in the T lymphocytes in the absence of a full-length NBS protein, presumably due to the presence of an alternatively transcribed NBS protein of 70 kDa. Normal protein expression patterns for pRb and p53 in all the immortal lines coincided with altered expression of some cell-cycle proteins as well as with an impaired G1/S arrest after gamma irradiation, despite a seemingly normal p53/p21 pathway. The here described, spontaneously immortalized NBS derived T-cell lines can be useful in future analysis of the biologic effects in the NBS.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Cell- och molekylärbiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Cell and Molecular Biology (hsv//eng)
Nyckelord
- Nijmegen Breakage syndrome
- T lymphocyte
- immortalization
- telomere biology
- cell cycle control
- Pathology
- Patologi
- patologi
- Pathology
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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