| Functional defect in neutrophil cytosols from two patients with autosomal recessive cytochrome-positive chronic granulomatous disease. | |
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MedLine Citation:
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PMID: 2539395 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The kinetics of activation of the respiratory burst oxidase in the cell-free oxidase-activating system have been explained by a three-stage mechanism in which the membrane-associated oxidase components M: (a) take up a cytosolic factor S to form a complex M.S that is (b) slowly converted in the second stage to a precatalytic species [M.S]*, which finally (c) takes up two more (possibly identical) cytosolic components, C alpha and C beta, to successively generate [M.S]*C alpha, a low-activity (i.e., high Km) oxidase, and finally [M.S]*C alpha C beta, the ordinary (i.e., low Km) oxidase (Babior, B.M., R. Kuver, and J.T. Curnutte. 1988. J. Biol. Chem. 263:1713-1718). Studies with the cell-free oxidase-activating system from normal neutrophils and from neutrophils obtained from two patients with type II (autosomal recessive cytochrome-positive) chronic granulomatous disease (CGD) have suggested that (a) the defective element in the cytosol from patient neutrophils is S; (b) in normal neutrophil cytosol, S is limiting with respect to M; and (c) C alpha and C beta interact cooperatively with the activated precursor complex [M.S]*. It was further speculated that S might be identical to the nonphosphorylated progenitor of the phosphorylated 48-kD proteins that are missing in certain forms of CGD, and that other forms of type II CGD besides the one described in this report remain to be discovered. |
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Authors:
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J T Curnutte; P J Scott; B M Babior |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Journal of clinical investigation Volume: 83 ISSN: 0021-9738 ISO Abbreviation: J. Clin. Invest. Publication Date: 1989 Apr |
Date Detail:
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Created Date: 1989-05-15 Completed Date: 1989-05-15 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 7802877 Medline TA: J Clin Invest Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1236-40 Citation Subset: AIM; IM |
Affiliation:
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Department of Basic and Clinical Research, Scripps Clinic, La Jolla, California 92037. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cell-Free System Cytosol / enzymology, physiology* Enzyme Activation Genes, Recessive Granulomatous Disease, Chronic / enzymology*, genetics, pathology Humans Kinetics NADH, NADPH Oxidoreductases / metabolism*, physiology NADPH Oxidase* Neutrophils / enzymology, physiology* Superoxides / biosynthesis* |
| Grant Support | |
ID/Acronym/Agency:
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AI-24227/AI/NIAID NIH HHS; AI-24838/AI/NIAID NIH HHS; RR-00833/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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11062-77-4/Superoxides; EC 1.6.-/NADH, NADPH Oxidoreductases; EC 1.6.3.1/NADPH Oxidase; EC 1.6.99.-/superoxide-forming enzyme |
| Comments/Corrections | |
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