Mouse model of X-linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production.

@article{citeulike:2961835, abstract = {Chronic granulomatous disease (CGD) is a recessive disorder characterized by a defective phagocyte respiratory burst oxidase, life-threatening pyogenic infections and inflammatory granulomas. Gene targeting was used to generate mice with a null allele of the gene involved in X-linked CGD, which encodes the 91 kD subunit of the oxidase cytochrome b. Affected hemizygous male mice lacked phagocyte superoxide production, manifested an increased susceptibility to infection with Staphylococcus aureus and Aspergillus fumigatus and had an altered inflammatory response in thioglycollate peritonitis. This animal model should aid in developing new treatments for CGD and in evaluating the role of phagocyte-derived oxidants in inflammation.}, address = {Department of Pediatrics, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana, USA.}, author = {Pollock, J. D. and Williams, D. A. and Gifford, M. A. and Li, L. L. and Du, X. and Fisherman, J. and Orkin, S. H. and Doerschuk, C. M. and Dinauer, M. C.}, citeulike-article-id = {2961835}, doi = {10.1038/ng0295-202}, issn = {1061-4036}, journal = {Nature genetics}, keywords = {immunodeficiency}, month = {February}, number = {2}, pages = {202--209}, posted-at = {2008-07-04 05:25:10}, priority = {2}, title = {Mouse model of X-linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production.}, url = {http://dx.doi.org/10.1038/ng0295-202}, volume = {9}, year = {1995} }

TY - JOUR ID - citeulike:2961835 L3 - citeulike-article-id:2961835 N2 - Chronic granulomatous disease (CGD) is a recessive disorder characterized by a defective phagocyte respiratory burst oxidase, life-threatening pyogenic infections and inflammatory granulomas. Gene targeting was used to generate mice with a null allele of the gene involved in X-linked CGD, which encodes the 91 kD subunit of the oxidase cytochrome b. Affected hemizygous male mice lacked phagocyte superoxide production, manifested an increased susceptibility to infection with Staphylococcus aureus and Aspergillus fumigatus and had an altered inflammatory response in thioglycollate peritonitis. This animal model should aid in developing new treatments for CGD and in evaluating the role of phagocyte-derived oxidants in inflammation. IS - 2 JF - Nature genetics SN - 1061-4036 AD - Department of Pediatrics, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana, USA. EP - 209 TI - Mouse model of X-linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production. VL - 9 SP - 202 KW - immunodeficiency AU - Pollock, JD AU - Williams, DA AU - Gifford, MA AU - Li, LL AU - Du, X AU - Fisherman, J AU - Orkin, SH AU - Doerschuk, CM AU - Dinauer, MC PY - 1995/02// UR - http://dx.doi.org/10.1038/ng0295-202 ER -