2a1x

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{{Seed}}
 
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[[Image:2a1x.png|left|200px]]
 
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==Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate==
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The line below this paragraph, containing "STRUCTURE_2a1x", creates the "Structure Box" on the page.
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<StructureSection load='2a1x' size='340' side='right'caption='[[2a1x]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<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>
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or leave the SCENE parameter empty for the default display.
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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]] 2.5&#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=AKG:2-OXOGLUTARIC+ACID'>AKG</scene>, <scene name='pdbligand=FE2:FE+(II)+ION'>FE2</scene></td></tr>
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{{STRUCTURE_2a1x| PDB=2a1x | SCENE= }}
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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=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>
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</table>
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== Disease ==
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[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>
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== Function ==
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[https://www.uniprot.org/uniprot/PAHX_HUMAN PAHX_HUMAN] Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
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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/a1/2a1x_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=2a1x ConSurf].
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<div style="clear:both"></div>
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===Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate===
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==See Also==
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*[[Dioxygenase 3D structures|Dioxygenase 3D structures]]
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== References ==
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<references/>
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The line below this paragraph, {{ABSTRACT_PUBMED_16186124}}, adds the Publication Abstract to the page
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__TOC__
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(as it appears on PubMed at http://www.pubmed.gov), where 16186124 is the PubMed ID number.
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</StructureSection>
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{{ABSTRACT_PUBMED_16186124}}
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==About this Structure==
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2A1X is a 1 chain structure of 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].
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==Reference==
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<ref group="xtra">PMID:16186124</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Phytanoyl-CoA dioxygenase]]
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[[Category: Large Structures]]
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[[Category: Arrowsmith, C.]]
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[[Category: Arrowsmith C]]
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[[Category: Bunkoczi, G.]]
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[[Category: Bunkoczi G]]
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[[Category: Butler, D.]]
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[[Category: Butler D]]
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[[Category: Delft, F Von.]]
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[[Category: Edwards A]]
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[[Category: Edwards, A.]]
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[[Category: Kavanagh KL]]
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[[Category: Kavanagh, K L.]]
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[[Category: McDonough MA]]
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[[Category: McDonough, M A.]]
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[[Category: Oppermann U]]
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[[Category: Oppermann, U.]]
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[[Category: Schofield CJ]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Searles T]]
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[[Category: Schofield, C J.]]
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[[Category: Sundstrom M]]
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[[Category: Searles, T.]]
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[[Category: Von Delft F]]
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[[Category: Sundstrom, M.]]
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[[Category: Beta jelly roll]]
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[[Category: Double-stranded beta-helix]]
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[[Category: Sgc]]
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[[Category: Structural genomic]]
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[[Category: Structural genomics consortium]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 09:13:41 2009''
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Current revision

Human phytanoyl-coa 2-hydroxylase in complex with iron and 2-oxoglutarate

PDB ID 2a1x

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