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1. Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor

Author

Lebron, Jose A., Bennett, Melanie J., Vaughn, Daniel E., Chirino, Arthur J., Snow, Peter M, Mintier, Gabriel A., Feder, John N., and Bjorkman, Pamela J.

Subjects
Hemochromatosis -- Research, Proteins -- Structure, Biological sciences
Abstract

A study was conducted to determine the crystal structure of a soluble form of human HFE, a major histocompatibility complex-related protein that is mutated in the iron-overload disease hereditary hemochromatosis. The locations of hemochromatosis mutations and a patch of histidines that could be involved in pH-dependent interactions were observed in the structure of HFE. Also, tight binding of soluble transferrin receptor (TfR) and HFE was demonstrated at the basic pH of the cell surface. Findings support previous demonstration that HFE, transferrin and TfR form a ternary complex.

Published
1998

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