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Restoration of superoxide generation to a chronic granulomatous disease-
derived B-cell line by retrovirus mediated gene transfer
A Thrasher, M Chetty, C Casimir and AW Segal
Department of Medicine, Rayne Institute, University College London, UK.
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.
Volume 80,
Issue 5,
pp. 1125-1129,
09/01/1992
Copyright © 1992 by The American Society of Hematology
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