1a6z
From Proteopedia
(New page: 200px<br /> <applet load="1a6z" size="450" color="white" frame="true" align="right" spinBox="true" caption="1a6z, resolution 2.60Å" /> '''HFE (HUMAN) HEMOCHR...) |
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| - | [[Image:1a6z.gif|left|200px]]<br /> | + | [[Image:1a6z.gif|left|200px]]<br /><applet load="1a6z" size="350" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="1a6z" size=" | + | |
caption="1a6z, resolution 2.60Å" /> | caption="1a6z, resolution 2.60Å" /> | ||
'''HFE (HUMAN) HEMOCHROMATOSIS PROTEIN'''<br /> | '''HFE (HUMAN) HEMOCHROMATOSIS PROTEIN'''<br /> | ||
==Overview== | ==Overview== | ||
| - | HFE is an MHC-related protein that is mutated in the iron-overload disease | + | 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. |
==Disease== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1A6Z is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1A6Z is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1A6Z OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Protein complex]] | [[Category: Protein complex]] | ||
| - | [[Category: Bennett, M | + | [[Category: Bennett, M J.]] |
| - | [[Category: Bjorkman, P | + | [[Category: Bjorkman, P J.]] |
| - | [[Category: Chirino, A | + | [[Category: Chirino, A J.]] |
| - | [[Category: Feder, J | + | [[Category: Feder, J N.]] |
| - | [[Category: Lebron, J | + | [[Category: Lebron, J A.]] |
| - | [[Category: Mintier, G | + | [[Category: Mintier, G A.]] |
| - | [[Category: Snow, P | + | [[Category: Snow, P M.]] |
| - | [[Category: Vaughn, D | + | [[Category: Vaughn, D E.]] |
[[Category: hereditary hemochromatosis]] | [[Category: hereditary hemochromatosis]] | ||
[[Category: hfe]] | [[Category: hfe]] | ||
[[Category: mhc class i]] | [[Category: mhc class i]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 11:41:32 2008'' |
Revision as of 09:41, 21 February 2008
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HFE (HUMAN) HEMOCHROMATOSIS PROTEIN
Contents |
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.
Disease
Known diseases associated with this structure: Hemochromatosis OMIM:[235200], Hypoproteinemia, hypercatabolic OMIM:[109700], 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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