2jif
From Proteopedia
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- | {{Seed}} | ||
- | [[Image:2jif.png|left|200px]] | ||
- | + | ==Structure of human short-branched chain acyl-CoA dehydrogenase (ACADSB)== | |
- | + | <StructureSection load='2jif' size='340' side='right'caption='[[2jif]], [[Resolution|resolution]] 2.00Å' scene=''> | |
- | + | == Structural highlights == | |
- | + | <table><tr><td colspan='2'>[[2jif]] is a 4 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=2JIF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JIF 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]] 2Å</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=COS:COENZYME+A+PERSULFIDE'>COS</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</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=2jif FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jif OCA], [https://pdbe.org/2jif PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jif RCSB], [https://www.ebi.ac.uk/pdbsum/2jif PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jif ProSAT]</span></td></tr> | |
+ | </table> | ||
+ | == Disease == | ||
+ | [https://www.uniprot.org/uniprot/ACDSB_HUMAN ACDSB_HUMAN] 2-methylbutyryl-CoA dehydrogenase deficiency. The disease is caused by variants affecting the gene represented in this entry. | ||
+ | == Function == | ||
+ | [https://www.uniprot.org/uniprot/ACDSB_HUMAN ACDSB_HUMAN] Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (PubMed:7698750, PubMed:11013134, PubMed:21430231, PubMed:10832746). Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA (PubMed:7698750, PubMed:11013134, PubMed:21430231, PubMed:10832746). Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway (PubMed:11013134, PubMed:10832746). Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug (PubMed:8660691).<ref>PMID:10832746</ref> <ref>PMID:11013134</ref> <ref>PMID:21430231</ref> <ref>PMID:7698750</ref> <ref>PMID:8660691</ref> | ||
+ | == 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/ji/2jif_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=2jif ConSurf]. | ||
+ | <div style="clear:both"></div> | ||
- | === | + | ==See Also== |
- | + | *[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]] | |
- | + | == References == | |
- | == | + | <references/> |
- | + | __TOC__ | |
+ | </StructureSection> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
- | [[Category: | + | [[Category: Large Structures]] |
- | [[Category: | + | [[Category: Arrowsmith CH]] |
- | [[Category: Edwards | + | [[Category: Edwards A]] |
- | [[Category: Hozjan | + | [[Category: Hozjan V]] |
- | [[Category: Kavanagh | + | [[Category: Kavanagh KL]] |
- | [[Category: Niesen | + | [[Category: Niesen FH]] |
- | [[Category: Oppermann | + | [[Category: Oppermann U]] |
- | [[Category: Pike | + | [[Category: Pike ACW]] |
- | [[Category: Smee | + | [[Category: Smee C]] |
- | [[Category: Sundstrom | + | [[Category: Sundstrom M]] |
- | [[Category: Turnbull | + | [[Category: Turnbull AP]] |
- | [[Category: Umeano | + | [[Category: Umeano C]] |
- | [[Category: Weigelt | + | [[Category: Weigelt J]] |
- | [[Category: | + | [[Category: Von Delft F]] |
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Current revision
Structure of human short-branched chain acyl-CoA dehydrogenase (ACADSB)
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Categories: Homo sapiens | Large Structures | Arrowsmith CH | Edwards A | Hozjan V | Kavanagh KL | Niesen FH | Oppermann U | Pike ACW | Smee C | Sundstrom M | Turnbull AP | Umeano C | Weigelt J | Von Delft F