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

Abstract:

: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.

journal_name

Scand J Immunol

authors

de Oliveira-Junior EB,Bustamante J,Newburger PE,Condino-Neto A

doi

10.1111/j.1365-3083.2010.02501.x

subject

Has Abstract

pub_date

2011-05-01 00:00:00

pages

420-7

issue

5

eissn

0300-9475

issn

1365-3083

journal_volume

73

pub_type

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