Literature DB >> 1528026

Biochemistry and molecular biology of chronic granulomatous disease.

A W Segal1.   

Abstract

The NADPH oxidase of phagocytic cells is an electron transport chain in the wall of the endocytic vacuole that is activated upon engulfment of the microbe, and is important for its efficient killing and digestion. The oxidase consists of a heterodimeric cytochrome b in the membrane, which is the site of the haem and FAD groups, and two cystolic factors p47-phox and p67-phox that appear to activate the system. Absence of this oxidase as a result of defects in each of these specialized proteins causes the syndrome of chronic granulomatous disease (CGD), that is characterized by a profound predisposition to pyogenic infection.

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Year:  1992        PMID： 1528026     DOI： 10.1007/bf01799624

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  24 in total

1.  The alpha subunit of cytochrome b-245 mapped to chromosome 16.

Authors:  H N Bu-Ghanim; C M Casimir; S Povey; A W Segal
Journal:  Genomics       Date:  1990-11       Impact factor: 5.736

Review 2.  Non-catalytic domains of cytoplasmic protein-tyrosine kinases: regulatory elements in signal transduction.

Authors:  T Pawson
Journal:  Oncogene       Date:  1988-11       Impact factor: 9.867

3.  Characterization of the 47-kilodalton autosomal chronic granulomatous disease protein: tissue-specific expression and transcriptional control by retinoic acid.

Authors:  A R Rodaway; C G Teahan; C M Casimir; A W Segal; D L Bentley
Journal:  Mol Cell Biol       Date:  1990-10       Impact factor: 4.272

4.  Human neutrophil cytochrome b light chain (p22-phox). Gene structure, chromosomal location, and mutations in cytochrome-negative autosomal recessive chronic granulomatous disease.

Authors:  M C Dinauer; E A Pierce; G A Bruns; J T Curnutte; S H Orkin
Journal:  J Clin Invest       Date:  1990-11       Impact factor: 14.808

5.  Cloning of a 67-kD neutrophil oxidase factor with similarity to a noncatalytic region of p60c-src.

Authors:  T L Leto; K J Lomax; B D Volpp; H Nunoi; J M Sechler; W M Nauseef; R A Clark; J I Gallin; H L Malech
Journal:  Science       Date:  1990-05-11       Impact factor: 47.728

6.  Two cytosolic neutrophil oxidase components absent in autosomal chronic granulomatous disease.

Authors:  B D Volpp; W M Nauseef; R A Clark
Journal:  Science       Date:  1988-12-02       Impact factor: 47.728

7.  Cloning of the cDNA and functional expression of the 47-kilodalton cytosolic component of human neutrophil respiratory burst oxidase.

Authors:  B D Volpp; W M Nauseef; J E Donelson; D R Moser; R A Clark
Journal:  Proc Natl Acad Sci U S A       Date:  1989-09       Impact factor: 11.205

8.  Cytochrome b-245 is a flavocytochrome containing FAD and the NADPH-binding site of the microbicidal oxidase of phagocytes.

Authors:  A W Segal; I West; F Wientjes; J H Nugent; A J Chavan; B Haley; R C Garcia; H Rosen; G Scrace
Journal:  Biochem J       Date:  1992-06-15       Impact factor: 3.857

9.  The X-linked chronic granulomatous disease gene codes for the beta-chain of cytochrome b-245.

Authors:  C Teahan; P Rowe; P Parker; N Totty; A W Segal
Journal:  Nature       Date:  1987 Jun 25-Jul 1       Impact factor: 49.962

10.  Classification of chronic granulomatous disease.

Authors:  J T Curnutte
Journal:  Hematol Oncol Clin North Am       Date:  1988-06       Impact factor: 3.722
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