1mj4

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[[Image:1mj4.jpg|left|200px]]
 
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{{Structure
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==Crystal Structure Analysis of the cytochrome b5 domain of human sulfite oxidase==
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|PDB= 1mj4 |SIZE=350|CAPTION= <scene name='initialview01'>1mj4</scene>, resolution 1.20&Aring;
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<StructureSection load='1mj4' size='340' side='right'caption='[[1mj4]], [[Resolution|resolution]] 1.20&Aring;' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND= <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene> and <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>
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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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|ACTIVITY= [http://en.wikipedia.org/wiki/Sulfite_oxidase Sulfite oxidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.8.3.1 1.8.3.1]
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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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|GENE=
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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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}}
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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>
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'''Crystal Structure Analysis of the cytochrome b5 domain of human sulfite oxidase'''
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==See Also==
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*[[Sulfite Oxidase|Sulfite Oxidase]]
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== References ==
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==Overview==
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<references/>
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The molybdenum- and iron-containing enzyme sulfite oxidase catalyzes the physiologically vital oxidation of sulfite to sulfate. Sulfite oxidase contains three domains: an N-terminal cytochrome b(5) domain, a central domain harboring the molybdenum cofactor (Moco) and a C-terminal dimerization domain. Oxidation of the substrate sulfite is coupled to the transfer of two electrons to the molybdenum cofactor. Subsequently, these electrons are passed on, one at a time, to the b(5) heme of sulfite oxidase and from there to the soluble electron carrier cytochrome c. The crystal structure of the oxidized human sulfite oxidase cytochrome b(5) domain has been determined at 1.2 A resolution and has been refined to a crystallographic R factor of 0.107 (R(free) = 0.137). A comparison of this structure with other b(5)-type cytochromes reveals distinct structural features present in the sulfite oxidase b(5) domain which promote optimal electron transport between the Moco of sulfite oxidase and the heme of cytochrome c.
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__TOC__
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</StructureSection>
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==Disease==
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Known disease associated with this structure: Sulfite oxidase deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606887 606887]]
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==About this Structure==
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1MJ4 is a [[Single protein]] structure of 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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==Reference==
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The 1.2 A structure of the human sulfite oxidase cytochrome b(5) domain., Rudolph MJ, Johnson JL, Rajagopalan KV, Kisker C, Acta Crystallogr D Biol Crystallogr. 2003 Jul;59(Pt 7):1183-91. Epub 2003, Jun 27. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/12832761 12832761]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Sulfite oxidase]]
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[[Category: Johnson JL]]
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[[Category: Johnson, J L.]]
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[[Category: Kisker C]]
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[[Category: Kisker, C.]]
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[[Category: Rajagopalan KV]]
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[[Category: Rajagopalan, K V.]]
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[[Category: Rudolph MJ]]
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[[Category: Rudolph, M J.]]
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[[Category: GOL]]
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[[Category: HEM]]
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[[Category: SO4]]
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[[Category: cytochrome b5]]
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[[Category: heme]]
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[[Category: sulfite oxidase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 12:44:00 2008''
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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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