{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:18:09Z","timestamp":1791296289376,"version":"4.1.0"},"reference-count":31,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"4883","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[1988,12,2]]},"abstract":"<jats:p>\n            Chronic granulomatous diseases of childhood (CGD) are a group of disorders of phagocytic cell superoxide (O\n            <jats:sub>2<\/jats:sub>\n            \u22c5\n            <jats:sup>-<\/jats:sup>\n            ) 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 O\n            <jats:sub>2<\/jats:sub>\n            \u22c5\n            <jats:sup>-<\/jats:sup>\n            -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 O\n            <jats:sub>2<\/jats:sub>\n            \u22c5\n            <jats:sup>-<\/jats:sup>\n            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 O\n            <jats:sub>2<\/jats:sub>\n            \u22c5\n            <jats:sup>-<\/jats:sup>\n            by phagocytic cells and that genetic abnormalities of these cytosol components can result in the CGD phenotype.\n          <\/jats:p>","DOI":"10.1126\/science.2848319","type":"journal-article","created":{"date-parts":[[2006,10,5]],"date-time":"2006-10-05T21:21:15Z","timestamp":1160083275000},"page":"1298-1301","source":"Crossref","is-referenced-by-count":252,"title":["Two Forms of Autosomal Chronic Granulomatous Disease Lack Distinct Neutrophil Cytosol Factors"],"prefix":"10.1126","volume":"242","author":[{"given":"Hiroyuki","family":"Nunoi","sequence":"first","affiliation":[{"name":"Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Rotrosen","sequence":"additional","affiliation":[{"name":"Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John I.","family":"Gallin","sequence":"additional","affiliation":[{"name":"Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry L.","family":"Malech","sequence":"additional","affiliation":[{"name":"Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_2_1_1_1","first-page":"A40","volume":"18","year":"1988","unstructured":"BOLSCHER, BGJ, EUR J CLIN INVEST 18: A40 (1988).","journal-title":"EUR J CLIN INVEST"},{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1083\/jcb.97.1.52","volume":"97","year":"1983","unstructured":"BORREGAARD, N, SUBCELLULAR-LOCALIZATION OF THE B-CYTOCHROME COMPONENT OF THE HUMAN NEUTROPHIL MICROBICIDAL OXIDASE - 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