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. 1995 Sep 1;182(3):751-8.
doi: 10.1084/jem.182.3.751.

The p47phox mouse knock-out model of chronic granulomatous disease

S H Jackson  1 , J I Gallin, S M Holland
Affiliations

The p47phox mouse knock-out model of chronic granulomatous disease

S H Jackson et al. J Exp Med. .

Abstract

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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