1a6z

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[[Image:1a6z.gif|left|200px]]
[[Image:1a6z.gif|left|200px]]
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{{Structure
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|PDB= 1a6z |SIZE=350|CAPTION= <scene name='initialview01'>1a6z</scene>, resolution 2.60&Aring;
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The line below this paragraph, containing "STRUCTURE_1a6z", creates the "Structure Box" on the page.
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|GENE= HFE ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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{{STRUCTURE_1a6z| PDB=1a6z | SCENE= }}
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1a6z FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1a6z OCA], [http://www.ebi.ac.uk/pdbsum/1a6z PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1a6z RCSB]</span>
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'''HFE (HUMAN) HEMOCHROMATOSIS PROTEIN'''
'''HFE (HUMAN) HEMOCHROMATOSIS PROTEIN'''
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[[Category: Snow, P M.]]
[[Category: Snow, P M.]]
[[Category: Vaughn, D E.]]
[[Category: Vaughn, D E.]]
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[[Category: hereditary hemochromatosis]]
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[[Category: Hereditary hemochromatosis]]
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[[Category: hfe]]
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[[Category: Hfe]]
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[[Category: mhc class i]]
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[[Category: Mhc class i]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 09:54:29 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 18:34:36 2008''
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Revision as of 06:54, 2 May 2008

Template:STRUCTURE 1a6z

HFE (HUMAN) HEMOCHROMATOSIS PROTEIN


Overview

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.

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 Page seeded by OCA on Fri May 2 09:54:29 2008

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