Recombinant 47-Kilodalton Cytosol Factor Restores NADPH Oxidase in Chronic Granulomatous Disease
Open Access
- 28 July 1989
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 245 (4916), 409-412
- https://doi.org/10.1126/science.2547247
Abstract
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.Keywords
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