CHRONIC GRANULOMATOUS-DISEASE

被引：15

作者：

DINAUER, MC

ORKIN, SH

机构：

[1] HARVARD UNIV,SCH MED,DANA FARBER CANC INST,DEPT PEDIAT,BOSTON,MA 02115

[2] HOWARD HUGHES MED INST,BOSTON,MA 02115

来源：

ANNUAL REVIEW OF MEDICINE | 1992年 / 43卷

关键词：

INTERFERON-GAMMA; RESPIRATORY BURST; SUPEROXIDE; NADPH-OXIDASE; CYTOCHROME-B; PHAGOCYTE; NEUTROPHIL; MACROPHAGE;

D O I：

10.1146/annurev.med.43.1.117

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Chronic granulomatous disease (CGD) encompasses a group of rare inherited disorders characterized by defects in a phagocyte-specific NADPH-oxidase complex that forms the superoxide radical during the respiratory burst. In this chapter, the protein components and cellular biochemistry of the oxidase are reviewed in light of recent genetic and biochemical studies of CGD. The classification and molecular genetic analysis of CGD is discussed. Finally, the use of recombinant human interferon-gamma as a new therapeutic agent for management of the disorder is reviewed.

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页码：117 / 124

页数：8

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