The human NADPH oxidase: primary and secondary defects impairing the respiratory burst function and the microbicidal ability of phagocytes.

作者: EB de Oliveira‐Junior , J Bustamante , Peter E Newburger , Antonio Condino‐Neto , None

DOI: 10.1111/J.1365-3083.2010.02501.X

关键词: Respiratory burstPhagocyteImmunologyNADPH oxidaseMutationChronic granulomatous diseaseReactive oxygen speciesOxidase testSuperoxideBiology

摘要: Phagocytes, such as granulocytes and monocytes/macrophages, contain a membrane-associated NADPH oxidase that produces superoxide leading to other reactive oxygen species with microbicidal, tumoricidal inflammatory activities. Primary defects in activity chronic granulomatous disease (CGD) lead severe, life-threatening infections demonstrate the importance of oxygen-dependent microbicidal system host defence. Other immunological disturbances may secondarily affect system, impair phagocytes predispose recurrent infections. This article reviews primary human classical CGD, more recently discovered affecting phagocyte respiratory burst function resulting immunodeficiencies varied phenotypes, including susceptibilities pyogenic or mycobacterial

参考文章(94)
Mason Pj, New insights into G6PD deficiency. British Journal of Haematology. ,vol. 94, pp. 585- 591 ,(1996)
Georg Wilhelm Löhr, Hans Dierck Waller, Glucose-6-phosphate Dehydrogenase Methods of Enzymatic Analysis (Second Edition)#R##N#Volume 2. pp. 636- 643 ,(1974) , 10.1016/B978-0-12-091302-2.50026-8
R S Weening, A de Klein, D Roos, L Corbeel, M de Boer, R Seger, J P Hossle, Cytochrome b558-negative, autosomal recessive chronic granulomatous disease: two new mutations in the cytochrome b558 light chain of the NADPH oxidase (p22-phox). American Journal of Human Genetics. ,vol. 51, pp. 1127- 1135 ,(1992)
Beulah Holmes, PaulG. Quie, DorothyB. Windhorst, RobertA. Good, FATAL GRANULOMATOUS DISEASE OF CHILDHOOD The Lancet. ,vol. 287, pp. 1225- 1228 ,(1966) , 10.1016/S0140-6736(66)90238-8
Anjali Singh, Kol A. Zarember, Douglas B. Kuhns, John I. Gallin, Impaired priming and activation of the neutrophil NADPH oxidase in patients with IRAK4 or NEMO deficiency. Journal of Immunology. ,vol. 182, pp. 6410- 6417 ,(2009) , 10.4049/JIMMUNOL.0802512
Pablo J. Patiño, Julie Rae, Deborah Noack, Rich Erickson, Jiabing Ding, Diana Garcı́a de Olarte, John T. Curnutte, Molecular characterization of autosomal recessive chronic granulomatous disease caused by a defect of the nicotinamide adenine dinucleotide phosphate (reduced form) oxidase component p67-phox. Blood. ,vol. 94, pp. 2505- 2514 ,(1999) , 10.1182/BLOOD.V94.7.2505.419K10_2505_2514
D Roos, M de Boer, F Kuribayashi, C Meischl, RS Weening, AW Segal, A Ahlin, K Nemet, JP Hossle, E Bernatowska-Matuszkiewicz, H Middleton-Price, Mutations in the X-linked and autosomal recessive forms of chronic granulomatous disease Blood. ,vol. 87, pp. 1663- 1681 ,(1996) , 10.1182/BLOOD.V87.5.1663.1663