2f2s: Difference between revisions
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New page: left|200px<br /> <applet load="2f2s" size="450" color="white" frame="true" align="right" spinBox="true" caption="2f2s, resolution 2.00Å" /> '''Human mitochondrial... |
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== | ==Human mitochondrial acetoacetyl-CoA thiolase== | ||
<StructureSection load='2f2s' size='340' side='right'caption='[[2f2s]], [[Resolution|resolution]] 2.00Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[2f2s]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=2es8 2es8]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2F2S OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2F2S FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=COA:COENZYME+A'>COA</scene></td></tr> | |||
<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=SCY:S-ACETYL-CYSTEINE'>SCY</scene></td></tr> | |||
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ACAT1, ACAT, MAT ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> | |||
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/Acetyl-CoA_C-acetyltransferase Acetyl-CoA C-acetyltransferase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.3.1.9 2.3.1.9] </span></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=2f2s FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2f2s OCA], [https://pdbe.org/2f2s PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2f2s RCSB], [https://www.ebi.ac.uk/pdbsum/2f2s PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2f2s ProSAT]</span></td></tr> | |||
</table> | |||
== Disease == | |||
[[https://www.uniprot.org/uniprot/THIL_HUMAN THIL_HUMAN]] Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:[https://omim.org/entry/203750 203750]]; also known as alpha-methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype.<ref>PMID:1346617</ref> <ref>PMID:1715688</ref> <ref>PMID:7728148</ref> <ref>PMID:9744475</ref> | |||
== Function == | |||
[[https://www.uniprot.org/uniprot/THIL_HUMAN THIL_HUMAN]] Plays a major role in ketone body metabolism. | |||
== 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/f2/2f2s_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=2f2s ConSurf]. | |||
<div style="clear:both"></div> | |||
== | ==See Also== | ||
*[[Thiolase 3D structures|Thiolase 3D structures]] | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Acetyl-CoA C-acetyltransferase]] | [[Category: Acetyl-CoA C-acetyltransferase]] | ||
[[Category: | [[Category: Human]] | ||
[[Category: | [[Category: Large Structures]] | ||
[[Category: Antoshenko, T | [[Category: Antoshenko, T]] | ||
[[Category: Arrowsmith, C | [[Category: Arrowsmith, C H]] | ||
[[Category: Bochkarev, A | [[Category: Bochkarev, A]] | ||
[[Category: Dombrovski, L | [[Category: Dombrovski, L]] | ||
[[Category: Edwards, A | [[Category: Edwards, A M]] | ||
[[Category: Loppnau, P | [[Category: Loppnau, P]] | ||
[[Category: Min, J | [[Category: Min, J R]] | ||
[[Category: Plotnikov, A | [[Category: Plotnikov, A N]] | ||
[[Category: | [[Category: Structural genomic]] | ||
[[Category: Sundstrom, M | [[Category: Sundstrom, M]] | ||
[[Category: Weigelt, J | [[Category: Weigelt, J]] | ||
[[Category: Wu, H | [[Category: Wu, H]] | ||
[[Category: | [[Category: Acat1]] | ||
[[Category: | [[Category: Acetyl-coenzyme a acetyltransferase 1]] | ||
[[Category: | [[Category: Mat]] | ||
[[Category: | [[Category: Sgc]] | ||
[[Category: | [[Category: T2]] | ||
[[Category: | [[Category: Thil]] | ||
[[Category: Transferase]] | |||
Latest revision as of 10:37, 17 February 2021
Human mitochondrial acetoacetyl-CoA thiolase
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