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

Abstract:

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 sla mice, hephaestin was present only in the supranuclear compartment. In contrast, the iron permease Ireg1 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.

journal_name

Gut

journal_title

Gut

authors

Kuo YM,Su T,Chen H,Attieh Z,Syed BA,McKie AT,Anderson GJ,Gitschier J,Vulpe CD

doi

10.1136/gut.2003.019026

keywords:

subject

Has Abstract

pub_date

2004-02-01 00:00:00

pages

201-6

issue

2

eissn

0017-5749

issn

1468-3288

journal_volume

53

pub_type

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