J Clin Invest 55(4): 707-713
NADPH oxidase deficiency in X-linked chronic granulomatous disease.
Abstract
We measured the cyanide-insensitive pyridine nucleotide oxidase activity of fractionated resting and phagocytic neutrophils from 11 normal donors, 1 patient with hereditary deficiency of myeloperoxidase, and 7 patients with X-linked chronic granulomatous disease (CGD). When measured under optimal conditions (at pH 5.5 and in the presence of 0.5 mM Mn++), NADPH oxidase activity increased fourfold with phagocytosis and was six-fold higher than with NADH. Phagocytic neutrophils from patients with CGD were markedly deficient in NADPH oxidase activity.
Full text
Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.2M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.
Images in this article
Click on the image to see a larger version.
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- BRIDGES RA, BERENDES H, GOOD RA. A fatal granulomatous disease of childhood; the clinical, pathological, and laboratory features of a new syndrome. AMA J Dis Child. 1959 Apr;97(4):387–408. [PubMed] [Google Scholar]
- Holmes B, Quie PG, Windhorst DB, Good RA. Fatal granulomatous disease of childhood. An inborn abnormality of phagocytic function. Lancet. 1966 Jun 4;1(7449):1225–1228. [PubMed] [Google Scholar]
- Quie PG, White JG, Holmes B, Good RA. In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. J Clin Invest. 1967 Apr;46(4):668–679.[PMC free article] [PubMed] [Google Scholar]
- COHN ZA, MORSE SI. Functional and metabolic properties of polymorphonuclear leucocytes. I. Observations on the requirements and consequences of particle ingestion. J Exp Med. 1960 May 1;111:667–687.[PMC free article] [PubMed] [Google Scholar]
- SBARRA AJ, KARNOVSKY ML. The biochemical basis of phagocytosis. I. Metabolic changes during the ingestion of particles by polymorphonuclear leukocytes. J Biol Chem. 1959 Jun;234(6):1355–1362. [PubMed] [Google Scholar]
- STAHELIN H, KARNOVSKY ML, FARNHAM AE, SUTER E. Studies on the interaction between phagocytes and tubercle bacilli. III. Some metabolic effects in guinea pigs associated with infection with tubercle bacilli. J Exp Med. 1957 Mar 1;105(3):265–277.[PMC free article] [PubMed] [Google Scholar]
- Paul B, Sbarra AJ. The role of the phagocyte in host-parasite interactions. 13. The direct quantitative estimation of H2O2 in phagocytizing cells. Biochim Biophys Acta. 1968 Feb 1;156(1):168–178. [PubMed] [Google Scholar]
- Babior BM, Kipnes RS, Curnutte JT. Biological defense mechanisms. The production by leukocytes of superoxide, a potential bactericidal agent. J Clin Invest. 1973 Mar;52(3):741–744.[PMC free article] [PubMed] [Google Scholar]
- Baehner RL, Nathan DG. Quantitative nitroblue tetrazolium test in chronic granulomatous disease. N Engl J Med. 1968 May 2;278(18):971–976. [PubMed] [Google Scholar]
- Holmes B, Page AR, Good RA. Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocytic function. J Clin Invest. 1967 Sep;46(9):1422–1432.[PMC free article] [PubMed] [Google Scholar]
- CAGAN RH, KARNOVSKY ML. ENZYMATIC BASIS OF THE RESPIRATORY STIMULATION DURING PHAGOCYTOSIS. Nature. 1964 Oct 17;204:255–257. [PubMed] [Google Scholar]
- Baehner RL, Gilman N, Karnovsky ML. Respiration and glucose oxidation in human and guinea pig leukocytes: comparative studies. J Clin Invest. 1970 Apr;49(4):692–700.[PMC free article] [PubMed] [Google Scholar]
- Rossi F, Zatti M. Biochemical aspects of phagocytosis in polymorphonuclear leucocytes. NADH and NADPH oxidation by the granules of resting and phagocytizing cells. Experientia. 1964 Jan 15;20(1):21–23. [PubMed] [Google Scholar]
- Patriarca P, Cramer R, Moncalvo S, Rossi F, Romeo D. Enzymatic basis of metabolic stimulation in leucocytes during phagocytosis: the role of activated NADPH oxidase. Arch Biochem Biophys. 1971 Jul;145(1):255–262. [PubMed] [Google Scholar]
- IYER GY, QUESTEL JH. NADPH and NADH oxidation by guinea pig polymorphonuclear leucocytes. Can J Biochem Physiol. 1963 Feb;41:427–434. [PubMed] [Google Scholar]
- Paul BB, Strauss RR, Jacobs AA, Sbarra AJ. Direct involvement of NADPH oxidase with the stimulated respiratory and hexose monophosphate shunt activities in phagocytizing leukocytes. Exp Cell Res. 1972 Aug;73(2):456–462. [PubMed] [Google Scholar]
- BECK WS. Occurrence and control of the phosphogluconate oxidation pathway in normal and leukemic leukocytes. J Biol Chem. 1958 May;232(1):271–283. [PubMed] [Google Scholar]
- Böyum A. Isolation of mononuclear cells and granulocytes from human blood. Isolation of monuclear cells by one centrifugation, and of granulocytes by combining centrifugation and sedimentation at 1 g. Scand J Clin Lab Invest Suppl. 1968;97:77–89. [PubMed] [Google Scholar]
- LOWRY OH, ROSEBROUGH NJ, FARR AL, RANDALL RJ. Protein measurement with the Folin phenol reagent. J Biol Chem. 1951 Nov;193(1):265–275. [PubMed] [Google Scholar]
- Baehner RL, Karnovsky ML. Deficiency of reduced nicotinamide-adenine dinucleotide oxidase in chronic granulomatous disease. Science. 1968 Dec 13;162(3859):1277–1279. [PubMed] [Google Scholar]
- Rossi F, Romeo D, Patriarca P. Mechanism of phagocytosis-associated oxidative metabolism in polymorphonuclear leucocytes and macrophages. J Reticuloendothel Soc. 1972 Aug;12(2):127–149. [PubMed] [Google Scholar]
- ROBERTS J, QUASTEL JH. OXIDATION OF REDUCED TRIPHOSPHOPYRIDINE NUCLEOTIDE BY GUINEA PIG POLYMORPHONUCLEAR LEUCOCYTES. Nature. 1964 Apr 4;202:85–86. [PubMed] [Google Scholar]
- AKAZAWA T, CONN EE. The oxidation of reduced pyridine nucleotides by peroxidase. J Biol Chem. 1958 May;232(1):403–415. [PubMed] [Google Scholar]
- Karnovsky ML. Chronic granulomatous disease--pieces of a cellular and molecular puzzle. Fed Proc. 1973 Apr;32(4):1527–1533. [PubMed] [Google Scholar]