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The Human NADPH Oxidase: Primary and Secondary Defects Impairing the Respiratory Burst Function and the Microbicidal Ability of Phagocytes
Author(s) -
de OliveiraJunior E. B.,
Bustamante J.,
Newburger P. E.,
CondinoNeto A.
Publication year - 2011
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.2010.02501.x
Subject(s) - respiratory burst , nadph oxidase , primary (astronomy) , phagocyte , microbiology and biotechnology , oxidase test , respiratory system , immunology , biology , chemistry , phagocytosis , biochemistry , reactive oxygen species , enzyme , anatomy , physics , astronomy
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 and inflammatory activities. Primary defects in oxidase activity in chronic granulomatous disease (CGD) lead to severe, life‐threatening infections that demonstrate the importance of the oxygen‐dependent microbicidal system in host defence. Other immunological disturbances may secondarily affect the NADPH oxidase system, impair the microbicidal activity of phagocytes and predispose the host to recurrent infections. This article reviews the primary defects of the human NADPH oxidase leading to classical CGD, and more recently discovered immunological defects secondarily affecting phagocyte respiratory burst function and resulting in primary immunodeficiencies with varied phenotypes, including susceptibilities to pyogenic or mycobacterial infections.