Restoration of superoxide generation to a chronic granulomatous disease-derived B-cell line by retrovirus mediated gene transfer
Thrasher, Adrian J. and Chetty, M. and Casimir, Colin M. and Segal, Anthony W. (1992) Restoration of superoxide generation to a chronic granulomatous disease-derived B-cell line by retrovirus mediated gene transfer. Blood, 80 (5). pp. 1125-1129. ISSN 0006-4971
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Failure of a superoxide generating system, the NADPH oxidase, present in neutrophils and other phagocytes gives rise to chronic granulomatous disease (CGD), a group of single-gene inherited disorders all characterized by an extreme susceptibility to pyogenic infection, with potentially fatal consequences. About 30% of CGD cases are caused by an autosomally inherited deficiency of a 47-Kd cytoplasmic component of the oxidase (p47-phox). Epstein-Barr virus (EBV) immortalized B-lymphocyte lines established from these CGD patients also express this NADPH oxidase defect and consequently are rendered incapable of generating superoxide on stimulation. We have used a p47-phox-deficient EBV-transformed B-cell line as a recipient for retroviral transfer of a functional p47-phox cDNA. The presence and activity of the retrovirally encoded p47-phox in the transduced cells is demonstrated and we show that this restores their capacity to generate superoxide.
|Research Areas:||School of Science and Technology > Natural Sciences|
School of Science and Technology > Natural Sciences > Molecular Biology group
|Citations on ISI Web of Science:||50|
|Deposited On:||02 Dec 2009 09:42|
|Last Modified:||09 Oct 2014 11:56|
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