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

Nature Genetics volume 9, pages 202–209 (1995)Cite this article

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.

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Author information

Authors and Affiliations

  1. Herman B Wells Center for Pediatric Research, Departments of Pediatrics and of Medical and Molecular Genetics, James Whitcomb Riley Hospital for Children, Indiana University Medical Center,

    Jonathan D. Pollock, David A. Williams, Mary A.C. Gifford, Ling Lin Li, Xunxiang Du, Claire M. Doerschuk & Mary C. Dinauer

  2. Howard Hughes Medical Institute, Indiana University School of Medicine, Indianapolis, Indiana, 46202, USA

    David A. Williams

  3. Division of Hematology/Oncology, Children's Hospital and the Dana Farber Cancer Institute, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, 02115, USA

    Jason Fisherman & Stuart H. Orkin

  4. Howard Hughes Medical Institute, Boston, Massachusetts, 02115, USA

    Stuart H. Orkin

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Pollock, J., Williams, D., Gifford, M. et al. Mouse model of X–linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production. Nat Genet 9, 202–209 (1995). https://doi.org/10.1038/ng0295-202

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