2qd5

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{{STRUCTURE_2qd5| PDB=2qd5 | SCENE= }}
 
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===Structure of wild type human ferrochelatase in complex with a lead-porphyrin compound===
 
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{{ABSTRACT_PUBMED_17884090}}
 
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==Disease==
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==Structure of wild type human ferrochelatase in complex with a lead-porphyrin compound==
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[[http://www.uniprot.org/uniprot/HEMH_HUMAN HEMH_HUMAN]] Defects in FECH are the cause of erythropoietic protoporphyria (EPP) [MIM:[http://omim.org/entry/177000 177000]]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals.<ref>PMID:1755842</ref><ref>PMID:1376018</ref><ref>PMID:7910885</ref><ref>PMID:8757534</ref><ref>PMID:9585598</ref><ref>PMID:9740232</ref><ref>PMID:10942404</ref><ref>PMID:11375302</ref><ref>PMID:12063482</ref><ref>PMID:12601550</ref><ref>PMID:15286165</ref><ref>PMID:17196862</ref>
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<StructureSection load='2qd5' size='340' side='right'caption='[[2qd5]], [[Resolution|resolution]] 2.30&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[2qd5]] is a 2 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=2QD5 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2QD5 FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/HEMH_HUMAN HEMH_HUMAN]] Catalyzes the ferrous insertion into protoporphyrin IX.
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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]] 2.3&#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=ACY:ACETIC+ACID'>ACY</scene>, <scene name='pdbligand=CHD:CHOLIC+ACID'>CHD</scene>, <scene name='pdbligand=FES:FE2/S2+(INORGANIC)+CLUSTER'>FES</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=OXY:OXYGEN+MOLECULE'>OXY</scene>, <scene name='pdbligand=PB:LEAD+(II)+ION'>PB</scene>, <scene name='pdbligand=PP9:PROTOPORPHYRIN+IX'>PP9</scene></td></tr>
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==About this Structure==
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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=2qd5 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2qd5 OCA], [https://pdbe.org/2qd5 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2qd5 RCSB], [https://www.ebi.ac.uk/pdbsum/2qd5 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2qd5 ProSAT]</span></td></tr>
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[[2qd5]] is a 2 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=2QD5 OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/HEMH_HUMAN HEMH_HUMAN] Defects in FECH are the cause of erythropoietic protoporphyria (EPP) [MIM:[https://omim.org/entry/177000 177000]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals.<ref>PMID:1755842</ref> <ref>PMID:1376018</ref> <ref>PMID:7910885</ref> <ref>PMID:8757534</ref> <ref>PMID:9585598</ref> <ref>PMID:9740232</ref> <ref>PMID:10942404</ref> <ref>PMID:11375302</ref> <ref>PMID:12063482</ref> <ref>PMID:12601550</ref> <ref>PMID:15286165</ref> <ref>PMID:17196862</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/HEMH_HUMAN HEMH_HUMAN] Catalyzes the ferrous insertion into protoporphyrin IX.
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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/qd/2qd5_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=2qd5 ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[Ferrochelatase|Ferrochelatase]]
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*[[Ferrochelatase 3D structures|Ferrochelatase 3D structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:017884090</ref><references group="xtra"/><references/>
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__TOC__
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[[Category: Ferrochelatase]]
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Dailey, H A.]]
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[[Category: Large Structures]]
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[[Category: Dailey, T A.]]
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[[Category: Dailey HA]]
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[[Category: Lanzilotta, W N.]]
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[[Category: Dailey TA]]
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[[Category: Meldock, A E.]]
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[[Category: Lanzilotta WN]]
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[[Category: Ross, T A.]]
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[[Category: Meldock AE]]
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[[Category: Ferrochelatase]]
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[[Category: Ross TA]]
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[[Category: Heme synthesis]]
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[[Category: Lyase]]
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[[Category: Protoporphyrin ix]]
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Current revision

Structure of wild type human ferrochelatase in complex with a lead-porphyrin compound

PDB ID 2qd5

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