title: Defect in pyridine nucleotide dependent superoxide production by a particulate fraction from the granulocytes of patients with chronic granulomatous disease.
abstract: Particulate fractions from normal human granulocytes preactivated with opsonized zymosan were found to catalyze superoxide production in the presence of reduced pyridine nucleotides. Similar preparations from three patients with X-linked chronic granulomatous disease produced no detectable superoxide. The failure to produce superoxide was not due to an inhibitor, since cell-free preparations from the patients' granulocytes had no effect on superoxide production by normal particles. Particles from the mothers of two of the patients produced superoxide at diminished rates; superoxide production by particles from the third mother was normal. These findings suggest that chronic granulomatous disease represents either a defect in a pyridine nucleotide-dependent superoxide-forming oxidase or a lesion in the apparatus responsible for activating the oxidase.
mesh_terms: D002474:Cell-Free System; D002675:Child, Preschool; D003579:Cytochrome Reductases; D005260:Female; D006098:Granulocytes / Q000378:metabolism*; D006105:Granulomatous Disease, Chronic / Q000097:blood* / Q000201:enzymology / Q000235:genetics; D006801:Humans; D007962:Leukocytes / Q000378:metabolism*; D008297:Male; D008722:Methods; D009243:NAD; D009247:NADH, NADPH Oxidoreductases / Q000378:metabolism; D009249:NADP; D009895:Opsonin Proteins; D010084:Oxidation-Reduction; D010100:Oxygen / Q000096:biosynthesis*; D010585:Phagocyte Bactericidal Dysfunction / Q000097:blood*; D012730:Sex Chromosomes; D013481:Superoxides / Q000096:biosynthesis*; D015054:Zymosan / Q000378:metabolism
chemical_list: D009895:Opsonin Proteins; D009243:NAD; D013481:Superoxides; D009249:NADP; D015054:Zymosan; D009247:NADH, NADPH Oxidoreductases; D003579:Cytochrome Reductases; D010100:Oxygen