PMID- 14724150 OWN - NLM STAT- MEDLINE DCOM- 20040227 LR - 20190501 IS - 0017-5749 (Print) IS - 1458-3288 (Electronic) IS - 0017-5749 (Linking) VI - 53 IP - 2 DP - 2004 Feb TI - Mislocalisation of hephaestin, a multicopper ferroxidase involved in basolateral intestinal iron transport, in the sex linked anaemia mouse. PG - 201-6 AB - 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. FAU - Kuo, Y M AU - Kuo YM AD - Departments of Medicine and Pediatrics, University of California-San Francisco, San Francisco, CA 94143-0794, USA. FAU - Su, T AU - Su T FAU - Chen, H AU - Chen H FAU - Attieh, Z AU - Attieh Z FAU - Syed, B A AU - Syed BA FAU - McKie, A T AU - McKie AT FAU - Anderson, G J AU - Anderson GJ FAU - Gitschier, J AU - Gitschier J FAU - Vulpe, C D AU - Vulpe CD LA - eng GR - R01 DK 047192/DK/NIDDK NIH HHS/United States GR - 5R01 DK 57800/DK/NIDDK NIH HHS/United States GR - R01 DK056376/DK/NIDDK NIH HHS/United States GR - K08 DK 2823/DK/NIDDK NIH HHS/United States GR - 5R01 DK 56376/DK/NIDDK NIH HHS/United States GR - R01 DK057800/DK/NIDDK NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - England TA - Gut JT - Gut JID - 2985108R RN - 0 (HEPH protein, human) RN - 0 (Heph protein, mouse) RN - 0 (Membrane Proteins) RN - E1UOL152H7 (Iron) SB - IM MH - Anemia, Iron-Deficiency/genetics/*metabolism MH - Animals MH - Base Sequence MH - Biological Transport MH - Cell Membrane/chemistry MH - Cells, Cultured MH - *Duodenum MH - Enterocytes/chemistry MH - Humans MH - Intestinal Mucosa/*chemistry MH - Intracellular Fluid/chemistry MH - Iron/*metabolism MH - Membrane Proteins/*analysis/genetics MH - Mice MH - Mice, Inbred C57BL MH - Mice, Inbred Strains MH - Molecular Sequence Data PMC - PMC1774920 EDAT- 2004/01/16 05:00 MHDA- 2004/02/28 05:00 PMCR- 2007/02/01 CRDT- 2004/01/16 05:00 PHST- 2004/01/16 05:00 [pubmed] PHST- 2004/02/28 05:00 [medline] PHST- 2004/01/16 05:00 [entrez] PHST- 2007/02/01 00:00 [pmc-release] AID - 0530201 [pii] AID - 10.1136/gut.2003.019026 [doi] PST - ppublish SO - Gut. 2004 Feb;53(2):201-6. doi: 10.1136/gut.2003.019026.