| First Author | Jackson SH | Year | 1995 |
| Journal | J Exp Med | Volume | 182 |
| Issue | 3 | Pages | 751-8 |
| PubMed ID | 7650482 | Mgi Jnum | J:28267 |
| Mgi Id | MGI:75890 | Doi | 10.1084/jem.182.3.751 |
| Citation | Jackson SH, et al. (1995) The p47phox mouse knock-out model of chronic granulomatous disease. J Exp Med 182(3):751-8 |
| abstractText | 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. |
