2cw6: Difference between revisions

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{{Seed}}
[[Image:2cw6.png|left|200px]]


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==Crystal Structure of Human HMG-CoA Lyase: Insights into Catalysis and the Molecular Basis for Hydroxymethylglutaric Aciduria==
The line below this paragraph, containing "STRUCTURE_2cw6", creates the "Structure Box" on the page.
<StructureSection load='2cw6' size='340' side='right'caption='[[2cw6]], [[Resolution|resolution]] 2.10&Aring;' scene=''>
You may change the PDB parameter (which sets the PDB file loaded into the applet)
== Structural highlights ==
or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
<table><tr><td colspan='2'>[[2cw6]] is a 6 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=2CW6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2CW6 FirstGlance]. <br>
or leave the SCENE parameter empty for the default display.
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.1&#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=3HG:3-HYDROXYPENTANEDIOIC+ACID'>3HG</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></td></tr>
{{STRUCTURE_2cw6|  PDB=2cw6  |  SCENE=  }}
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2cw6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2cw6 OCA], [https://pdbe.org/2cw6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2cw6 RCSB], [https://www.ebi.ac.uk/pdbsum/2cw6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2cw6 ProSAT]</span></td></tr>
 
</table>
===Crystal Structure of Human HMG-CoA Lyase: Insights into Catalysis and the Molecular Basis for Hydroxymethylglutaric Aciduria===
== Disease ==
 
[https://www.uniprot.org/uniprot/HMGCL_HUMAN HMGCL_HUMAN] Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:[https://omim.org/entry/246450 246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.<ref>PMID:8798725</ref> <ref>PMID:9463337</ref> <ref>PMID:9784232</ref> <ref>PMID:11129331</ref> <ref>PMID:12746442</ref> <ref>PMID:17173698</ref> <ref>PMID:16601870</ref> <ref>PMID:17459752</ref> <ref>PMID:19036343</ref> <ref>PMID:19177531</ref>
 
== Function ==
<!--
[https://www.uniprot.org/uniprot/HMGCL_HUMAN HMGCL_HUMAN] Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism.<ref>PMID:8566388</ref> <ref>PMID:22865860</ref> <ref>PMID:22847177</ref>
The line below this paragraph, {{ABSTRACT_PUBMED_16330550}}, adds the Publication Abstract to the page
== Evolutionary Conservation ==
(as it appears on PubMed at http://www.pubmed.gov), where 16330550 is the PubMed ID number.
[[Image:Consurf_key_small.gif|200px|right]]
-->
Check<jmol>
{{ABSTRACT_PUBMED_16330550}}
  <jmolCheckbox>
 
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/cw/2cw6_consurf.spt"</scriptWhenChecked>
==About this Structure==
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
2CW6 is a [[Single protein]] 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=2CW6 OCA].  
    <text>to colour the structure by Evolutionary Conservation</text>
 
  </jmolCheckbox>
==Reference==
</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=2cw6 ConSurf].
Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase: insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria., Fu Z, Runquist JA, Forouhar F, Hussain M, Hunt JF, Miziorko HM, Kim JJ, J Biol Chem. 2006 Mar 17;281(11):7526-32. Epub 2005 Dec 5. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/16330550 16330550]
<div style="clear:both"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Hydroxymethylglutaryl-CoA lyase]]
[[Category: Large Structures]]
[[Category: Single protein]]
[[Category: Fu Z]]
[[Category: Fu, Z.]]
[[Category: Hunt JF]]
[[Category: Hunt, J F.]]
[[Category: Kim J-JP]]
[[Category: Kim, J J.P.]]
[[Category: Miziorko HM]]
[[Category: Miziorko, H M.]]
[[Category: Runquist JA]]
[[Category: Runquist, J A.]]
[[Category: Hmg-coa lyase]]
[[Category: Ketogenic enzyme]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 00:24:42 2008''
Retrieved from "https://proteopedia.org/2cw6"