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[[Image:3ecr.png|left|200px]]


{{STRUCTURE_3ecr|  PDB=3ecr  |  SCENE=  }}
==Structure of human porphobilinogen deaminase==
 
<StructureSection load='3ecr' size='340' side='right'caption='[[3ecr]], [[Resolution|resolution]] 2.18&Aring;' scene=''>
===Structure of human porphobilinogen deaminase===
== Structural highlights ==
 
<table><tr><td colspan='2'>[[3ecr]] 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=3ECR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3ECR FirstGlance]. <br>
{{ABSTRACT_PUBMED_18936296}}
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.182&#8491;</td></tr>
 
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=DPM:3-[5-{[3-(2-CARBOXYETHYL)-4-(CARBOXYMETHYL)-5-METHYL-1H-PYRROL-2-YL]METHYL}-4-(CARBOXYMETHYL)-1H-PYRROL-3-YL]PROPANOIC+ACID'>DPM</scene></td></tr>
==About this Structure==
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3ecr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3ecr OCA], [https://pdbe.org/3ecr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3ecr RCSB], [https://www.ebi.ac.uk/pdbsum/3ecr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3ecr ProSAT]</span></td></tr>
[[3ecr]] 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=3ECR OCA].  
</table>
== Disease ==
[https://www.uniprot.org/uniprot/HEM3_HUMAN HEM3_HUMAN] Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:[https://omim.org/entry/176000 176000]. AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.<ref>PMID:2243128</ref> <ref>PMID:1714233</ref> <ref>PMID:1496994</ref> <ref>PMID:1427766</ref> <ref>PMID:1301948</ref> <ref>PMID:8262523</ref> <ref>PMID:8401516</ref> <ref>PMID:8268934</ref> <ref>PMID:8270254</ref> <ref>PMID:8270256</ref> <ref>PMID:8081367</ref> <ref>PMID:7962538</ref> <ref>PMID:7757070</ref> <ref>PMID:8825929</ref> <ref>PMID:9199558</ref> <ref>PMID:9225970</ref> <ref>PMID:9654202</ref> <ref>PMID:9463797</ref> <ref>PMID:10494093</ref> <ref>PMID:10453740</ref> <ref>PMID:10502788</ref> <ref>PMID:10657149</ref> <ref>PMID:10602775</ref> <ref>PMID:11399210</ref> <ref>PMID:11030413</ref> <ref>PMID:10782018</ref> <ref>PMID:11013452</ref> [:]<ref>PMID:12406973</ref> <ref>PMID:12372055</ref> <ref>PMID:11857754</ref> <ref>PMID:14669009</ref> <ref>PMID:14970743</ref> <ref>PMID:15669678</ref>
== Function ==
[https://www.uniprot.org/uniprot/HEM3_HUMAN HEM3_HUMAN] Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/ec/3ecr_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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=3ecr ConSurf].
<div style="clear:both"></div>


==See Also==
==See Also==
*[[Porphobilinogen Deaminase|Porphobilinogen Deaminase]]
*[[Porphobilinogen Deaminase|Porphobilinogen Deaminase]]
 
== References ==
==Reference==
<references/>
<ref group="xtra">PMID:018936296</ref><references group="xtra"/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Hydroxymethylbilane synthase]]
[[Category: Large Structures]]
[[Category: Cheng, C.]]
[[Category: Cheng C]]
[[Category: Gao, A.]]
[[Category: Gao A]]
[[Category: Joachimiak, A.]]
[[Category: Joachimiak A]]
[[Category: Li, Y.]]
[[Category: Li Y]]
[[Category: Liu, Z J.]]
[[Category: Liu ZJ]]
[[Category: Shaw, N.]]
[[Category: Shaw N]]
[[Category: Song, G.]]
[[Category: Song G]]
[[Category: Zhang, R.]]
[[Category: Zhang R]]
[[Category: Zhao, Y.]]
[[Category: Zhao Y]]
[[Category: Disease mutation]]
[[Category: Heme biosynthesis]]
[[Category: Human porphobilinogen deaminase]]
[[Category: Porphobilinogen hinge]]
[[Category: Porphyrin biosynthesis]]
[[Category: Transferase]]

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