1mj4

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{{STRUCTURE_1mj4| PDB=1mj4 | SCENE= }}
 
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===Crystal Structure Analysis of the cytochrome b5 domain of human sulfite oxidase===
 
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{{ABSTRACT_PUBMED_12832761}}
 
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==Disease==
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==Crystal Structure Analysis of the cytochrome b5 domain of human sulfite oxidase==
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[[http://www.uniprot.org/uniprot/SUOX_HUMAN SUOX_HUMAN]] Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD) [MIM:[http://omim.org/entry/272300 272300]]; also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.<ref>PMID:9428520</ref><ref>PMID:9600976</ref><ref>PMID:10519592</ref><ref>PMID:12112661</ref><ref>PMID:12368985</ref>
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<StructureSection load='1mj4' size='340' side='right'caption='[[1mj4]], [[Resolution|resolution]] 1.20&Aring;' scene=''>
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== Structural highlights ==
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==About this Structure==
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<table><tr><td colspan='2'>[[1mj4]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1MJ4 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1MJ4 FirstGlance]. <br>
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[[1mj4]] is a 1 chain structure with sequence 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=1MJ4 OCA].
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.2&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1mj4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1mj4 OCA], [https://pdbe.org/1mj4 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1mj4 RCSB], [https://www.ebi.ac.uk/pdbsum/1mj4 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1mj4 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/SUOX_HUMAN SUOX_HUMAN] Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD) [MIM:[https://omim.org/entry/272300 272300]; also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.<ref>PMID:9428520</ref> <ref>PMID:9600976</ref> <ref>PMID:10519592</ref> <ref>PMID:12112661</ref> <ref>PMID:12368985</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/SUOX_HUMAN SUOX_HUMAN]
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/mj/1mj4_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1mj4 ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
*[[Sulfite Oxidase|Sulfite Oxidase]]
*[[Sulfite Oxidase|Sulfite Oxidase]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:012832761</ref><ref group="xtra">PMID:012207032</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Sulfite oxidase]]
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[[Category: Large Structures]]
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[[Category: Johnson, J L.]]
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[[Category: Johnson JL]]
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[[Category: Kisker, C.]]
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[[Category: Kisker C]]
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[[Category: Rajagopalan, K V.]]
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[[Category: Rajagopalan KV]]
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[[Category: Rudolph, M J.]]
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[[Category: Rudolph MJ]]
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[[Category: Cytochrome b5]]
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[[Category: Heme]]
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[[Category: Oxidoreductase]]
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[[Category: Sulfite oxidase]]
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Current revision

Crystal Structure Analysis of the cytochrome b5 domain of human sulfite oxidase

PDB ID 1mj4

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