title: Mouse model of X-linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production.
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.
authors.affiliation: Department of Pediatrics, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana, USA.
authors.forename: J D
authors.initials: JD
authors.lastname: Pollock
authors.forename: D A
authors.initials: DA
authors.lastname: Williams
authors.forename: M A
authors.initials: MA
authors.lastname: Gifford
authors.forename: L L
authors.initials: LL
authors.lastname: Li
authors.forename: X
authors.initials: X
authors.lastname: Du
authors.forename: J
authors.initials: J
authors.lastname: Fisherman
authors.forename: S H
authors.initials: SH
authors.lastname: Orkin
authors.forename: C M
authors.initials: CM
authors.lastname: Doerschuk
authors.forename: M C
authors.initials: MC
authors.lastname: Dinauer
mesh_terms: D000483:Alleles; D000818:Animals; D001228:Aspergillosis; D001232:Aspergillus fumigatus; D003573:Cytochrome b Group / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D005260:Female; D008040:Genetic Linkage; D006105:Granulomatous Disease, Chronic / Q000235:genetics* / Q000503:physiopathology; D008172:Lung Diseases, Fungal; D008264:Macrophages / Q000201:enzymology; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic / Q000235:genetics* / Q000502:physiology; D009504:Neutrophils / Q000201:enzymology; D010538:Peritonitis / Q000139:chemically induced; D010586:Phagocytes / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D013203:Staphylococcal Infections; D013211:Staphylococcus aureus; D013234:Stem Cells / Q000502:physiology; D013481:Superoxides / Q000378:metabolism*; D014960:X Chromosome
chemical_list: D003573:Cytochrome b Group; D013481:Superoxides