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Science 2 December 1988:
Vol. 242. no. 4883, pp. 1298 - 1301
DOI: 10.1126/science.2848319
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Articles

Science, Vol 242, Issue 4883, 1298-1301
Copyright © 1988 by American Association for the Advancement of Science

articles

Two forms of autosomal chronic granulomatous disease lack distinct neutrophil cytosol factors

H Nunoi, D Rotrosen, JI Gallin, and HL Malech

Bacterial Diseases Section, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892.

Chronic granulomatous diseases of childhood (CGD) are a group of disorders of phagocytic cell superoxide (O2.-) production (respiratory burst). Anion exchange chromatography separated from normal neutrophil cytosol a 47-kilodalton neutrophil cytosol factor, NCF-1, that restored activity to defective neutrophil cytosol from most patients with autosomally inherited CGD in a cell-free O2.--generating system. A 65-kilodalton factor, NCF-2, restored activity to defective neutrophil cytosol from one patient with autosomal CGD. NCF-1, NCF-2, and a third cytosol fraction, NCF-3, were inactive alone or in pairs, but together replaced unfractionated cytosol in cell-free O2.- generation. Neutrophils deficient in NCF-1, but not NCF-2, did not phosphorylate the 47-kilodalton protein. It is proposed that NCF-1, NCF-2, and NCF-3 are essential for generation of O2.- by phagocytic cells and that genetic abnormalities of these cytosol components can result in the CGD phenotype.


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Science. ISSN 0036-8075 (print), 1095-9203 (online)