2w8p

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{{Seed}}
 
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[[Image:2w8p.jpg|left|200px]]
 
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==The crystal structure of human C340A SSADH==
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The line below this paragraph, containing "STRUCTURE_2w8p", creates the "Structure Box" on the page.
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<StructureSection load='2w8p' size='340' side='right'caption='[[2w8p]], [[Resolution|resolution]] 2.30&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'>[[2w8p]] 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=2W8P OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2W8P 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]] 2.3&#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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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{{STRUCTURE_2w8p| PDB=2w8p | 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=2w8p FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2w8p OCA], [https://pdbe.org/2w8p PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2w8p RCSB], [https://www.ebi.ac.uk/pdbsum/2w8p PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2w8p ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/SSDH_HUMAN SSDH_HUMAN] Defects in ALDH5A1 are the cause of succinate semialdehyde dehydrogenase deficiency (SSADH deficiency) [MIM:[https://omim.org/entry/271980 271980]. SSADH deficiency is a rare inborn error in the metabolism of 4-aminobutyric acid (GABA) which leads to accumulation of 4-hydroxybutyric acid in physiologic fluids of patients. The disease is characterized by severe ataxia and by mildly retarded psychomotor development.
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== Function ==
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[https://www.uniprot.org/uniprot/SSDH_HUMAN SSDH_HUMAN] Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).<ref>PMID:19300440</ref>
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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/w8/2w8p_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=2w8p ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Succinic semialdehyde dehydrogenase (SSADH) is involved in the final degradation step of the inhibitory neurotransmitter gamma-aminobutyric acid by converting succinic semialdehyde to succinic acid in the mitochondrial matrix. SSADH deficiency, a rare autosomal recessive disease, exhibits variable clinical phenotypes, including psychomotor retardation, language delay, behaviour disturbance and convulsions. Here, we present crystal structures of both the oxidized and reduced forms of human SSADH. Interestingly, the structures show that the catalytic loop of the enzyme undergoes large structural changes depending on the redox status of the environment, which is mediated by a reversible disulphide bond formation between a catalytic Cys340 and an adjacent Cys342 residues located on the loop. Subsequent in vivo and in vitro studies reveal that the 'dynamic catalytic loop' confers a response to reactive oxygen species and changes in redox status, indicating that the redox-switch modulation could be a physiological control mechanism of human SSADH. Structural basis for the substrate specificity of the enzyme and the impact of known missense point mutations associated with the disease pathogenesis are presented as well.
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===THE CRYSTAL STRUCTURE OF HUMAN C340A SSADH===
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Redox-switch modulation of human SSADH by dynamic catalytic loop.,Kim YG, Lee S, Kwon OS, Park SY, Lee SJ, Park BJ, Kim KJ EMBO J. 2009 Apr 8;28(7):959-68. Epub 2009 Mar 19. PMID:19300440<ref>PMID:19300440</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 2w8p" style="background-color:#fffaf0;"></div>
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==See Also==
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The line below this paragraph, {{ABSTRACT_PUBMED_19300440}}, adds the Publication Abstract to the page
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*[[Aldehyde dehydrogenase 3D structures|Aldehyde dehydrogenase 3D structures]]
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(as it appears on PubMed at http://www.pubmed.gov), where 19300440 is the PubMed ID number.
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*[[Succinate-semialdehyde dehydrogenase|Succinate-semialdehyde dehydrogenase]]
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== References ==
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{{ABSTRACT_PUBMED_19300440}}
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<references/>
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__TOC__
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==About this Structure==
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</StructureSection>
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2W8P 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=2W8P OCA].
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==Reference==
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<ref group="xtra">PMID:19300440</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Succinate-semialdehyde dehydrogenase]]
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[[Category: Large Structures]]
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[[Category: Kim, K J.]]
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[[Category: Kim K-J]]
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[[Category: Kim, Y G.]]
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[[Category: Kim Y-G]]
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[[Category: Disease mutation]]
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[[Category: Mitochondria]]
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[[Category: Mitochondrion]]
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[[Category: Nad]]
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[[Category: Oxidoreductase]]
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[[Category: Polymorphism]]
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[[Category: Ssa]]
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[[Category: Ssadh]]
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[[Category: Transit peptide]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jun 10 17:56:29 2009''
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Current revision

The crystal structure of human C340A SSADH

PDB ID 2w8p

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