Interactions of the ectodomain of HFE with the transferrin receptor are critical for iron homeostasis in cells

FEBS Letters

Enns, Caroline A.²  Basava, Alivelu¹  Feder, John N.¹  Starnes, Steven M.¹  Carlson, Eric J.²  Anderson, Emily L.²  Roy, Cindy N.² 

[1]Progenitor Inc., Menlo Park, CA 94025, USA

[2]Department of Cell and Developmental Biology, L215, Oregon Health Sciences University, Portland, OR 97201-3098, USA

关键词: Hemochromatosis;    HFE;    Iron metabolism;    Ferritin;    Transferrin receptor;   

DOI  :  10.1016/S0014-5793(00)02173-6

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Expression of wild type HFE reduces the ferritin levels of cells in culture. In this report we demonstrate that the predominant hereditary hemochromatosis mutation, C282Y² HFE, does not reduce ferritin expression. However, the second mutation, H63D HFE, reduces ferritin expression to a level indistinguishable from cells expressing wild type HFE. Further, two HFE cytoplasmic domain mutations engineered to disrupt potential signal transduction, S335M and Y342C, were functionally indistinguishable from wild type HFE in this assay, as was soluble HFE. These results implicate a role for the interaction of HFE with the transferrin receptor in lowering cellular ferritin levels.

【 授权许可】

Unknown