Chronic granulomatous disease

Mary C. Dinauer; Stuart H. Orkin

doi:10.1146/annurev.me.43.020192.001001

Chronic granulomatous disease

Mary C. Dinauer, Stuart H. Orkin

Research output: Contribution to journal › Review article › peer-review

90 Scopus citations

Abstract

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.

Original language	English
Pages (from-to)	117-124
Number of pages	8
Journal	Annual review of medicine
Volume	43
DOIs	https://doi.org/10.1146/annurev.me.43.020192.001001
State	Published - 1992

Keywords

NADPH-oxidase
cytochrome b
interferon-gamma
macrophage
neutrophil
phagocyte
respiratory burst
superoxide

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10.1146/annurev.me.43.020192.001001

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title = "Chronic granulomatous disease",

abstract = "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.",

keywords = "NADPH-oxidase, cytochrome b, interferon-gamma, macrophage, neutrophil, phagocyte, respiratory burst, superoxide",

author = "Dinauer, {Mary C.} and Orkin, {Stuart H.}",

year = "1992",

doi = "10.1146/annurev.me.43.020192.001001",

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volume = "43",

pages = "117--124",

journal = "Annual review of medicine",

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T1 - Chronic granulomatous disease

AU - Dinauer, Mary C.

AU - Orkin, Stuart H.

PY - 1992

Y1 - 1992

N2 - 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.

AB - 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.

KW - NADPH-oxidase

KW - cytochrome b

KW - interferon-gamma

KW - macrophage

KW - neutrophil

KW - phagocyte

KW - respiratory burst

KW - superoxide

UR - http://www.scopus.com/inward/record.url?scp=0026628807&partnerID=8YFLogxK

U2 - 10.1146/annurev.me.43.020192.001001

DO - 10.1146/annurev.me.43.020192.001001

M3 - Review article

C2 - 1316094

AN - SCOPUS:0026628807

SN - 0066-4219

VL - 43

SP - 117

EP - 124

JO - Annual review of medicine

JF - Annual review of medicine

ER -

Chronic granulomatous disease

Abstract

Keywords

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