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Science 28 July 1989:
Vol. 245. no. 4916, pp. 409 - 412
DOI: 10.1126/science.2547247
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Articles

Science, Vol 245, Issue 4916, 409-412
Copyright © 1989 by American Association for the Advancement of Science

articles

Recombinant 47-kilodalton cytosol factor restores NADPH oxidase in chronic granulomatous disease

KJ Lomax, TL Leto, H Nunoi, JI Gallin, and HL Malech

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

A 47-kilodalton neutrophil cytosol factor (NCF-47k), required for activation of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase superoxide (O2-.) production, is absent in most patients with autosomal recessive chronic granulomatous disease (AR-CGD). NCF-47k cDNAs were cloned from an expression library. The largest clone predicted a 41.9-kD protein that contained an arginine and serine-rich COOH-terminal domain with potential protein kinase C phosphorylation sites. A 33-amino acid segment of NCF-47k shared 49% identity with ras p21 guanosine triphosphatase activating protein. Recombinant NCF-47k restored O2-. -producing activity to AR-CGD neutrophil cytosol in a cell-free assay. Production of active recombinant NCF-47k will enable functional regions of this molecule to be mapped.


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Cloning of a 67-kD neutrophil oxidase factor with similarity to a noncatalytic region of p60c-src.
T. Leto, K. Lomax, B. Volpp, H Nunoi, J. Sechler, W. Nauseef, R. Clark, J. Gallin, and H. Malech (1990)
Science 248, 727-730
   Abstract »    PDF »
Minor errors in two published sequences.
K. Lomax, T. Leto, H Nunoi, J. Gallin, and H. Malech (1989)
Science 246, 987
   PDF »


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