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.

journal_name

Nat Genet

journal_title

Nature genetics

authors

Pollock JD,Williams DA,Gifford MA,Li LL,Du X,Fisherman J,Orkin SH,Doerschuk CM,Dinauer MC

doi

10.1038/ng0295-202

subject

Has Abstract

pub_date

1995-02-01 00:00:00

pages

202-9

issue

2

eissn

1061-4036

issn

1546-1718

journal_volume

9

pub_type

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