1a6z

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{{Seed}}
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{{STRUCTURE_1a6z| PDB=1a6z | SCENE= }}
{{STRUCTURE_1a6z| PDB=1a6z | SCENE= }}
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'''HFE (HUMAN) HEMOCHROMATOSIS PROTEIN'''
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===HFE (HUMAN) HEMOCHROMATOSIS PROTEIN===
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==Overview==
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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.
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(as it appears on PubMed at http://www.pubmed.gov), where 9546397 is the PubMed ID number.
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{{ABSTRACT_PUBMED_9546397}}
==Disease==
==Disease==
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[[Category: Hfe]]
[[Category: Hfe]]
[[Category: Mhc class i]]
[[Category: Mhc class i]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Jun 20 09:30:39 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jun 30 16:16:38 2008''

Revision as of 13:16, 30 June 2008

Image:1a6z.png

Template:STRUCTURE 1a6z

Contents

HFE (HUMAN) HEMOCHROMATOSIS PROTEIN

Template:ABSTRACT PUBMED 9546397

Disease

Known disease associated with this structure: Hemochromatosis OMIM:[235200], Porphyria variegata OMIM:[235200]

About this Structure

1A6Z is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

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

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