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1a6z
From Proteopedia
Contents |
HFE (HUMAN) HEMOCHROMATOSIS PROTEIN
HFE is an MHC-related protein that is mutated in the iron-overload disease hereditary hemochromatosis. HFE binds to transferrin receptor (TfR) and reduces its affinity for iron-loaded transferrin, implicating HFE in iron metabolism. The 2.6 A crystal structure of HFE reveals the locations of hemochromatosis mutations and a patch of histidines that could be involved in pH-dependent interactions. We also demonstrate that soluble TfR and HFE bind tightly at the basic pH of the cell surface, but not at the acidic pH of intracellular vesicles. TfR:HFE stoichiometry (2:1) differs from TfR:transferrin stoichiometry (2:2), implying a different mode of binding for HFE and transferrin to TfR, consistent with our demonstration that HFE, transferrin, and TfR form a ternary complex.
Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor., Lebron JA, Bennett MJ, Vaughn DE, Chirino AJ, Snow PM, Mintier GA, Feder JN, Bjorkman PJ, Cell. 1998 Apr 3;93(1):111-23. PMID:9546397
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
Disease
Known disease associated with this structure: Hemochromatosis OMIM:[235200], Porphyria variegata OMIM:[235200]
About this Structure
1A6Z is a 4 chains structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Lebron JA, Bennett MJ, Vaughn DE, Chirino AJ, Snow PM, Mintier GA, Feder JN, Bjorkman PJ. Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor. Cell. 1998 Apr 3;93(1):111-23. PMID:9546397
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