Recombinant 47-kilodalton cytosol factor restores NADPH oxidase in chronic granulomatous disease
KJ Lomax, TL Leto, H Nunoi, JI Gallin, HL Malech - Science, 1989 - science.org
KJ Lomax, TL Leto, H Nunoi, JI Gallin, HL Malech
Science, 1989•science.orgA 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 …
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 …
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.
AAAS