Restoration of superoxide generation to a chronic granulomatous disease-derived B-cell line by retrovirus mediated gene transfer

Article

Thrasher, A., Chetty, M., Casimir, C. and Segal, A. 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.
TypeArticle
TitleRestoration of superoxide generation to a chronic granulomatous disease-derived B-cell line by retrovirus mediated gene transfer
AuthorsThrasher, A., Chetty, M., Casimir, C. and Segal, A.
Abstract

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 GroupMolecular Biology group
PublisherAmerican Society of Hematology
JournalBlood
ISSN0006-4971
Publication dates
PrintSep 1992
Publication process dates
Deposited02 Dec 2009
Output statusPublished
Web address (URL)http://bloodjournal.hematologylibrary.org/cgi/content/short/80/5/1125
LanguageEnglish
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