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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4883
|
pubmed:dateCreated |
1989-1-4
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pubmed:abstractText |
Chronic granulomatous diseases of childhood (CGD) are a group of disorders of phagocytic cell superoxide (O2.-) production (respiratory burst). Anion exchange chromatography separated from normal neutrophil cytosol a 47-kilodalton neutrophil cytosol factor, NCF-1, that restored activity to defective neutrophil cytosol from most patients with autosomally inherited CGD in a cell-free O2.--generating system. A 65-kilodalton factor, NCF-2, restored activity to defective neutrophil cytosol from one patient with autosomal CGD. NCF-1, NCF-2, and a third cytosol fraction, NCF-3, were inactive alone or in pairs, but together replaced unfractionated cytosol in cell-free O2.- generation. Neutrophils deficient in NCF-1, but not NCF-2, did not phosphorylate the 47-kilodalton protein. It is proposed that NCF-1, NCF-2, and NCF-3 are essential for generation of O2.- by phagocytic cells and that genetic abnormalities of these cytosol components can result in the CGD phenotype.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Dec
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pubmed:issn |
0036-8075
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
2
|
pubmed:volume |
242
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1298-301
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pubmed:dateRevised |
2007-3-19
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pubmed:meshHeading |
pubmed-meshheading:2848319-Blotting, Western,
pubmed-meshheading:2848319-Cell Membrane,
pubmed-meshheading:2848319-Cytosol,
pubmed-meshheading:2848319-Granulomatous Disease, Chronic,
pubmed-meshheading:2848319-Humans,
pubmed-meshheading:2848319-Molecular Weight,
pubmed-meshheading:2848319-Neutrophils,
pubmed-meshheading:2848319-Phosphoproteins,
pubmed-meshheading:2848319-Superoxides
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pubmed:year |
1988
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pubmed:articleTitle |
Two forms of autosomal chronic granulomatous disease lack distinct neutrophil cytosol factors.
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pubmed:affiliation |
Bacterial Diseases Section, National Institute of Allergy and Infectious Diseases, Bethesda, MD 20892.
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pubmed:publicationType |
Journal Article,
In Vitro
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