title: Two cytosolic neutrophil oxidase components absent in autosomal chronic granulomatous disease.
abstract: Neutrophils kill microorganisms with oxygen radicals generated by an oxidase that uses the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) as substrate. This system requires both membrane and cytosolic components and is defective in patients with chronic granulomatous disease. A cytosolic complex capable of activating latent membrane oxidase was eluted from guanosine triphosphate-agarose and was used to raise polyclonal antiserum that recognized 47- and 67-kilodalton proteins. These proteins were restricted to the cytosol of myeloid cells. Both proteins were associated with NADPH oxidase-activating capacity when neutrophil cytosol was purified on nucleotide affinity matrices or molecular sizing columns. Neutrophils from patients with two different forms of autosomal chronic granulomatous disease lacked either the 47- or 67-kilodalton protein.
authors.affiliation: Department of Medicine, University of Iowa, College of Medicine, Iowa City.
authors.forename: B D
authors.initials: BD
authors.lastname: Volpp
authors.forename: W M
authors.initials: WM
authors.lastname: Nauseef
authors.forename: R A
authors.initials: RA
authors.lastname: Clark
mesh_terms: D015153:Blotting, Western; D003600:Cytosol / Q000502:physiology; D019204:GTP-Binding Proteins / Q000502:physiology; D006105:Granulomatous Disease, Chronic / Q000201:enzymology*; D006801:Humans; D008970:Molecular Weight; D009247:NADH, NADPH Oxidoreductases / Q000172:deficiency*; D019255:NADPH Oxidases; D009504:Neutrophils / Q000201:enzymology*; D013481:Superoxides / Q000378:metabolism
chemical_list: D013481:Superoxides; D009247:NADH, NADPH Oxidoreductases; D019255:NADPH Oxidases; D019204:GTP-Binding Proteins