The p47phox mouse knock-out model of chronic granulomatous disease.

SH Jackson, JI Gallin, SM Holland - The Journal of experimental …, 1995 - rupress.org
SH Jackson, JI Gallin, SM Holland
The Journal of experimental medicine, 1995rupress.org
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 …
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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