2a1x: Difference between revisions

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-{{STRUCTURE_2a1x|  PDB=2a1x  |  SCENE=  }}
-===Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate===
-{{ABSTRACT_PUBMED_16186124}}
-==Disease==
+==Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate==
-[[http://www.uniprot.org/uniprot/PAHX_HUMAN PAHX_HUMAN]] Defects in PHYH are a cause of Refsum disease (RD) [MIM:[http://omim.org/entry/266500 266500]]. RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.<ref>PMID:9326939</ref><ref>PMID:9326940</ref><ref>PMID:10767344</ref><ref>PMID:10709665</ref>
+<StructureSection load='2a1x' size='340' side='right'caption='[[2a1x]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
+== Structural highlights ==
+<table><tr><td colspan='2'>[[2a1x]] 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=2A1X OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2A1X FirstGlance]. <br>
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.5&#8491;</td></tr>
+<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=AKG:2-OXOGLUTARIC+ACID'>AKG</scene>, <scene name='pdbligand=FE2:FE+(II)+ION'>FE2</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=2a1x FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2a1x OCA], [https://pdbe.org/2a1x PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2a1x RCSB], [https://www.ebi.ac.uk/pdbsum/2a1x PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2a1x ProSAT]</span></td></tr>
+</table>
+== Disease ==
+[https://www.uniprot.org/uniprot/PAHX_HUMAN PAHX_HUMAN] Defects in PHYH are a cause of Refsum disease (RD) [MIM:[https://omim.org/entry/266500 266500]. RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life.<ref>PMID:9326939</ref> <ref>PMID:9326940</ref> <ref>PMID:10767344</ref> <ref>PMID:10709665</ref>
+== Function ==
+[https://www.uniprot.org/uniprot/PAHX_HUMAN PAHX_HUMAN] Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
+== 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/a1/2a1x_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=2a1x ConSurf].
+<div style="clear:both"></div>
-==Function==
+==See Also==
-[[http://www.uniprot.org/uniprot/PAHX_HUMAN PAHX_HUMAN]] Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
+*[[Dioxygenase 3D structures|Dioxygenase 3D structures]]
+== References ==
-==About this Structure==
+<references/>
-[[2a1x]] is a 1 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=2A1X OCA].
+__TOC__
+</StructureSection>
-==Reference==
-<ref group="xtra">PMID:016186124</ref><references group="xtra"/><references/>
 [[Category: Homo sapiens]]
-[[Category: Phytanoyl-CoA dioxygenase]]
+[[Category: Large Structures]]
-[[Category: Arrowsmith, C.]]
+[[Category: Arrowsmith C]]
-[[Category: Bunkoczi, G.]]
+[[Category: Bunkoczi G]]
-[[Category: Butler, D.]]
+[[Category: Butler D]]
-[[Category: Delft, F Von.]]
+[[Category: Edwards A]]
-[[Category: Edwards, A.]]
+[[Category: Kavanagh KL]]
-[[Category: Kavanagh, K L.]]
+[[Category: McDonough MA]]
-[[Category: McDonough, M A.]]
+[[Category: Oppermann U]]
-[[Category: Oppermann, U.]]
+[[Category: Schofield CJ]]
-[[Category: SGC, Structural Genomics Consortium.]]
+[[Category: Searles T]]
-[[Category: Schofield, C J.]]
+[[Category: Sundstrom M]]
-[[Category: Searles, T.]]
+[[Category: Von Delft F]]
-[[Category: Sundstrom, M.]]
-[[Category: Beta jelly roll]]
-[[Category: Double-stranded beta-helix]]
-[[Category: Oxidoreductase]]
-[[Category: Sgc]]
-[[Category: Structural genomic]]
-[[Category: Structural genomics consortium]]