1a6z: Difference between revisions

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New page: left|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="450" color="white" frame="true" align="right" spinBox="true"  
caption="1a6z, resolution 2.60&Aring;" />
caption="1a6z, resolution 2.60&Aring;" />
'''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, 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.
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://ispc.weizmann.ac.il/oca-bin/ocashort?id=1A6Z OCA].  
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.J.]]
[[Category: Bennett, M J.]]
[[Category: Bjorkman, P.J.]]
[[Category: Bjorkman, P J.]]
[[Category: Chirino, A.J.]]
[[Category: Chirino, A J.]]
[[Category: Feder, J.N.]]
[[Category: Feder, J N.]]
[[Category: Lebron, J.A.]]
[[Category: Lebron, J A.]]
[[Category: Mintier, G.A.]]
[[Category: Mintier, G A.]]
[[Category: Snow, P.M.]]
[[Category: Snow, P M.]]
[[Category: Vaughn, D.E.]]
[[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://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 15:56:18 2007''
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 11:41:32 2008''