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Research Article Free access | 10.1172/JCI113153

Recombinant interferon gamma augments phagocyte superoxide production and X-chronic granulomatous disease gene expression in X-linked variant chronic granulomatous disease.

R A Ezekowitz, S H Orkin, and P E Newburger

Division of Hematology/Oncology, Children's Hospital, Boston, Massachusetts 02115.

Find articles by Ezekowitz, R. in: JCI | PubMed | Google Scholar

Division of Hematology/Oncology, Children's Hospital, Boston, Massachusetts 02115.

Find articles by Orkin, S. in: JCI | PubMed | Google Scholar

Division of Hematology/Oncology, Children's Hospital, Boston, Massachusetts 02115.

Find articles by Newburger, P. in: JCI | PubMed | Google Scholar

Published October 1, 1987 - More info

Published in Volume 80, Issue 4 on October 1, 1987
J Clin Invest. 1987;80(4):1009–1016. https://doi.org/10.1172/JCI113153.
© 1987 The American Society for Clinical Investigation
Published October 1, 1987 - Version history
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Abstract

We examined the potential of interferon gamma (IFN-gamma) to ameliorate the physiologic defect of chronic granulomatous disease (CGD) by studying its effects on CGD phagocyte superoxide generation, NADPH oxidase kinetics, cytochrome b559 content, and expression of X-CGD (the gene for the X-linked disease). Granulocytes and macrophages from three patients in two kindreds with "variant" X-linked CGD (i.e., with very low, but detectable, baseline superoxide-generating activity) responded to IFN-gamma with enhanced nitroblue tetrazolium reduction and two- to eightfold increases in superoxide generation. IFN-gamma did not augment the respiratory burst activity of phagocytes from patients with "classic" CGD (i.e., no detectable baseline superoxide generation) or autosomal variant CGD. Incubation of a responding patient's granulocytes with IFN-gamma nearly doubled the maximal velocity for the NADPH oxidase, but did not change its abnormal Michaelis constant. Although the interferon-treated CGD granulocytes produced superoxide at a rate 40% of normal, the cytochrome b spectrum remained undetectable. IFN-gamma treatment of cultured monocytes from an IFN-gamma-responsive CGD patient increased the steady state level of RNA transcripts from the X-CGD gene from barely detectable up to approximately 5% of normal.

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