Journal
JOURNAL OF INFECTIOUS DISEASES
Volume 194, Issue 8, Pages 1135-1140Publisher
UNIV CHICAGO PRESS
DOI: 10.1086/507705
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Funding
- NIAID NIH HHS [K08 AI059072] Funding Source: Medline
- NIDDK NIH HHS [R01 DK59010] Funding Source: Medline
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Fabry disease is an X-linked recessive disorder in which affected persons lack alpha-galactosidase A (alpha- GalA), which leads to excess glycosphingolipids in tissues, mainly globotriaosylceramide (Gb3). Gb3 is the cellular receptor for Shiga toxin (Stx), the primary virulence factor of enterohemorrhagic Escherichia coli. alpha-GalA-knockout mice were significantly protected against lethal intraperitoneal doses of Stx2 or oral doses of Stx2-expressing bacteria, compared with wild-type (wt) control mice. Kidneys of moribund wt mice revealed tubular necrosis, but no histopathologic changes were observed in Gb3-overexpressing mice. Reducing Gb3 levels in alpha-GalA-knockout mice by the intravenous injection of recombinant human a- GalA restored the susceptibility of knockout mice to lethal doses of Stx2. These results suggest that excess amounts of Gb3 in alpha-GalA-deficient mice may impair toxin delivery to susceptible tissues.
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