Recombinant 47-kilodalton cytosol factor restores NADPH oxidase in chronic granulomatous disease.
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Lomax KJ, Leto TL, Nunoi H, Gallin JI, Malech HL
Recombinant 47-kilodalton cytosol factor restores NADPH oxidase in chronic granulomatous disease.
Science. 1989 Jul 28;245(4916):409-12.
- PubMed ID
- 2547247 [ View in PubMed]
- 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.