Mislocalisation of hephaestin, a multicopper ferroxidase involved in basolateral intestinal iron transport, in the sex linked anaemia mouse

Kuo, Y. M.; Su, T.; Chen, H.; Attieh, Z.; Syed, B. A.; McKie, A. T.; Anderson, G. J.; Gitschier, J.; Vulpe, C. D.
February 2004
Gut;Feb2004, Vol. 53 Issue 2, p201
Academic Journal
Background: Hephaestin is a multicopper ferroxidase required for basolateral transport of iron from enterocytes. Sex linked anaemia (sla) mice have a defect in the release of iron from intestinal enterocytes into the circulation due to an interstitial deletion in the hephaestin gene (heph). Results: We have demonstrated that hephaestin is primarily localised to a supranuclear compartment in both intestinal enterocytes and in cultured cells. In normal intestinal enterocytes, hephaestin was also present on the basolateral surface. In sic mice, hephaestin was present only in the supranuclear compartment. In contrast, the iron permease Iregi localised to the basolateral membrane in both control and sla mice. Conclusion: We suggest that mislocalisation of hephaestin likely contributes to the functional defect in sla intestinal epithelium.


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