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''' | ==Crystal structure of human dihydroorotate dehydrogenase (DHODH) with DH03A367== | ||
<StructureSection load='4rr4' size='340' side='right' caption='[[4rr4]], [[Resolution|resolution]] 2.38Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[4rr4]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4RR4 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4RR4 FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=3V1:2-CHLORO-N-[3-(4-{[(2Z)-2-CYANO-3-CYCLOPROPYL-3-HYDROXYPROP-2-ENOYL]AMINO}PHENOXY)PHENYL]-4-METHYL-1,3-THIAZOLE-5-CARBOXAMIDE'>3V1</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=ORO:OROTIC+ACID'>ORO</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> | |||
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Dihydroorotate_dehydrogenase_(quinone) Dihydroorotate dehydrogenase (quinone)], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.3.5.2 1.3.5.2] </span></td></tr> | |||
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4rr4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4rr4 OCA], [http://pdbe.org/4rr4 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4rr4 RCSB], [http://www.ebi.ac.uk/pdbsum/4rr4 PDBsum]</span></td></tr> | |||
[[ | </table> | ||
== Disease == | |||
[[http://www.uniprot.org/uniprot/PYRD_HUMAN PYRD_HUMAN]] Defects in DHODH are the cause of postaxial acrofacial dysostosis (POADS) [MIM:[http://omim.org/entry/263750 263750]]; also known as Miller syndrome. POADS is characterized by severe micrognathia, cleft lip and/or palate, hypoplasia or aplasia of the posterior elements of the limbs, coloboma of the eyelids and supernumerary nipples. POADS is a very rare disorder: only 2 multiplex families, each consisting of 2 affected siblings born to unaffected, nonconsanguineous parents, have been described among a total of around 30 reported cases.<ref>PMID:19915526</ref> | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/PYRD_HUMAN PYRD_HUMAN]] Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor. | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Li, H]] | [[Category: Li, H]] | ||
[[Category: Ren, X]] | |||
[[Category: Zhu, J]] | [[Category: Zhu, J]] | ||
[[Category: Zhu, L]] | [[Category: Zhu, L]] | ||
[[Category: Fmn binding]] | |||
[[Category: Mitochondria inner membrane]] | |||
[[Category: Oxidoreductase]] | |||
[[Category: Oxidoreductase-oxidoreductase inhibitor complex]] | |||
Revision as of 07:41, 1 December 2015
Crystal structure of human dihydroorotate dehydrogenase (DHODH) with DH03A367Crystal structure of human dihydroorotate dehydrogenase (DHODH) with DH03A367
Structural highlights
Disease[PYRD_HUMAN] Defects in DHODH are the cause of postaxial acrofacial dysostosis (POADS) [MIM:263750]; also known as Miller syndrome. POADS is characterized by severe micrognathia, cleft lip and/or palate, hypoplasia or aplasia of the posterior elements of the limbs, coloboma of the eyelids and supernumerary nipples. POADS is a very rare disorder: only 2 multiplex families, each consisting of 2 affected siblings born to unaffected, nonconsanguineous parents, have been described among a total of around 30 reported cases.[1] Function[PYRD_HUMAN] Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor. References
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