caa a22 4500 469044632 CHVBK 20180323132820.0 cr unu---uuuuu 170328e19920701xx s 000 0 eng 10.1007/BF01799624 doi (NATIONALLICENCE)springer-10.1007/BF01799624 Segal A. Department of Medicine, University College London, Rayne Institute, University Street, WC1E 6JJ, London, UK aut Biochemistry and molecular biology of chronic granulomatous disease [Elektronische Daten] [A. Segal] Summary: The NADPH oxidase of phagocytic cells is an electron transport chain in the wall of the endocytic vacuole that is activated upon engulfment of the microbe, and is important for its efficient killing and digestion. The oxidase consists of a heterodimeric cytochrome b in the membrane, which is the site of the haem and FAD groups, and two cystolic factors p47-phox and p67-phox that appear to activate the system. Absence of this oxidase as a result of defects in each of these specialized proteins causes the syndrome of chronic granulomatous disease (CGD), that is characterized by a profound predisposition to pyogenic infection. SSIEM and Kluwer Academic Publishers, 1992 Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/4(1992-07-01), 683-686 0141-8955 15:4<683 1992 15 10545 https://doi.org/10.1007/BF01799624 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01799624 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Segal A. Department of Medicine, University College London, Rayne Institute, University Street, WC1E 6JJ, London, UK aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/4(1992-07-01), 683-686 0141-8955 15:4<683 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer