2aex: Difference between revisions

==The 1.58A Crystal Structure of Human Coproporphyrinogen Oxidase Reveals the Structural Basis of Hereditary Coproporphyria==

<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CIT:CITRIC+ACID'>CIT</scene></td></tr>

<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2aex FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2aex OCA], [https://pdbe.org/2aex PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2aex RCSB], [https://www.ebi.ac.uk/pdbsum/2aex PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2aex ProSAT]</span></td></tr>

[https://www.uniprot.org/uniprot/HEM6_HUMAN HEM6_HUMAN] Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:[https://omim.org/entry/121300 121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity.<ref>PMID:8012360</ref> <ref>PMID:7849704</ref> <ref>PMID:7757079</ref> <ref>PMID:9048920</ref> <ref>PMID:8990017</ref> <ref>PMID:9298818</ref> <ref>PMID:9888388</ref> <ref>PMID:12181641</ref> <ref>PMID:15896662</ref> <ref>PMID:16398658</ref>  

== Function ==

[https://www.uniprot.org/uniprot/HEM6_HUMAN HEM6_HUMAN] Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III.

== Evolutionary Conservation ==

[[Image:Consurf_key_small.gif|200px|right]]

    <text>to colour the structure by Evolutionary Conservation</text>

</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=2aex ConSurf].

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<references/>

</StructureSection>

[[Category: Bodnarova M]]

[[Category: Demeler B]]

[[Category: Flachsova E]]

[[Category: Lee DS]]

[[Category: Martasek P]]

[[Category: Raman CS]]