The p47phox mouse knock-out model of chronic granulomatous disease

Chronic granulomatous disease (CGD) is caused by a congenital defect in phagocyte reduced nicotinamide dinucleotide phosphate (NADPH) oxidase production of superoxide and related species. It is characterized by recurrent life-threatening bacterial and fungal infections and tissue granuloma formation...

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Detalles Bibliográficos
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 1995
Materias:
Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2192153/
https://www.ncbi.nlm.nih.gov/pubmed/7650482
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collection PubMed
description Chronic granulomatous disease (CGD) is caused by a congenital defect in phagocyte reduced nicotinamide dinucleotide phosphate (NADPH) oxidase production of superoxide and related species. It is characterized by recurrent life-threatening bacterial and fungal infections and tissue granuloma formation. We have created a mouse model of CGD by targeted disruption of p47phox, one of the genes in which mutations cause human CGD. Identical to the case in human CGD, leukocytes from p47phox-/- mice produced no superoxide and killed staphylococci ineffectively. p47phox-/- mice developed lethal infections and granulomatous inflammation similar to those encountered in human CGD patients. This model mirrors human CGD and confirms a critical role for the phagocyte NADPH oxidase in mammalian host defense.
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spelling pubmed-21921532008-04-16 The p47phox mouse knock-out model of chronic granulomatous disease J Exp Med Articles Chronic granulomatous disease (CGD) is caused by a congenital defect in phagocyte reduced nicotinamide dinucleotide phosphate (NADPH) oxidase production of superoxide and related species. It is characterized by recurrent life-threatening bacterial and fungal infections and tissue granuloma formation. We have created a mouse model of CGD by targeted disruption of p47phox, one of the genes in which mutations cause human CGD. Identical to the case in human CGD, leukocytes from p47phox-/- mice produced no superoxide and killed staphylococci ineffectively. p47phox-/- mice developed lethal infections and granulomatous inflammation similar to those encountered in human CGD patients. This model mirrors human CGD and confirms a critical role for the phagocyte NADPH oxidase in mammalian host defense. The Rockefeller University Press 1995-09-01 /pmc/articles/PMC2192153/ /pubmed/7650482 Text en This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Articles
The p47phox mouse knock-out model of chronic granulomatous disease
title The p47phox mouse knock-out model of chronic granulomatous disease
title_full The p47phox mouse knock-out model of chronic granulomatous disease
title_fullStr The p47phox mouse knock-out model of chronic granulomatous disease
title_full_unstemmed The p47phox mouse knock-out model of chronic granulomatous disease
title_short The p47phox mouse knock-out model of chronic granulomatous disease
title_sort p47phox mouse knock-out model of chronic granulomatous disease
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2192153/
https://www.ncbi.nlm.nih.gov/pubmed/7650482

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