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==Crystal structure of human glutamine synthetase in complex with ADP and phosphate==
==Crystal structure of human glutamine synthetase in complex with ADP and phosphate==
<StructureSection load='2ojw' size='340' side='right' caption='[[2ojw]], [[Resolution|resolution]] 2.05&Aring;' scene=''>
<StructureSection load='2ojw' size='340' side='right'caption='[[2ojw]], [[Resolution|resolution]] 2.05&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2ojw]] is a 5 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2OJW OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2OJW FirstGlance]. <br>
<table><tr><td colspan='2'>[[2ojw]] is a 5 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=2OJW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2OJW FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene></td></tr>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.05&#8491;</td></tr>
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">GLUL, GLNS ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene></td></tr>
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Glutamate--ammonia_ligase Glutamate--ammonia ligase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.3.1.2 6.3.1.2] </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=2ojw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ojw OCA], [https://pdbe.org/2ojw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2ojw RCSB], [https://www.ebi.ac.uk/pdbsum/2ojw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2ojw ProSAT]</span></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2ojw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ojw OCA], [http://pdbe.org/2ojw PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2ojw RCSB], [http://www.ebi.ac.uk/pdbsum/2ojw PDBsum]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/GLNA_HUMAN GLNA_HUMAN]] Defects in GLUL are the cause of congenital systemic glutamine deficiency (CSGD) [MIM:[http://omim.org/entry/610015 610015]]. CSGD is a rare developmental disorder with severe brain malformation resulting in multi-organ failure and neonatal death. Glutamine is largely absent from affected patients serum, urine and cerebrospinal fluid.<ref>PMID:16267323</ref>
[https://www.uniprot.org/uniprot/GLNA_HUMAN GLNA_HUMAN] Defects in GLUL are the cause of congenital systemic glutamine deficiency (CSGD) [MIM:[https://omim.org/entry/610015 610015]. CSGD is a rare developmental disorder with severe brain malformation resulting in multi-organ failure and neonatal death. Glutamine is largely absent from affected patients serum, urine and cerebrospinal fluid.<ref>PMID:16267323</ref>  
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/GLNA_HUMAN GLNA_HUMAN]] This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.<ref>PMID:18662667</ref>
[https://www.uniprot.org/uniprot/GLNA_HUMAN GLNA_HUMAN] This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.<ref>PMID:18662667</ref>  
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
Check<jmol>
   <jmolCheckbox>
   <jmolCheckbox>
     <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/oj/2ojw_consurf.spt"</scriptWhenChecked>
     <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/oj/2ojw_consurf.spt"</scriptWhenChecked>
     <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
     <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
     <text>to colour the structure by Evolutionary Conservation</text>
     <text>to colour the structure by Evolutionary Conservation</text>
   </jmolCheckbox>
   </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/chain_selection.php?pdb_ID=2ata ConSurf].
</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=2ojw ConSurf].
<div style="clear:both"></div>
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
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==See Also==
==See Also==
*[[Glutamine Synthase|Glutamine Synthase]]
*[[Glutamine synthetase 3D structures|Glutamine synthetase 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Glutamate--ammonia ligase]]
[[Category: Homo sapiens]]
[[Category: Human]]
[[Category: Large Structures]]
[[Category: Arrowsmith, C]]
[[Category: Arrowsmith C]]
[[Category: Berg, S Van Den]]
[[Category: Berglund H]]
[[Category: Berglund, H]]
[[Category: Busam RD]]
[[Category: Busam, R D]]
[[Category: Collins R]]
[[Category: Collins, R]]
[[Category: Edwards A]]
[[Category: Edwards, A]]
[[Category: Flodin S]]
[[Category: Flodin, S]]
[[Category: Flores A]]
[[Category: Flores, A]]
[[Category: Graslund S]]
[[Category: Graslund, S]]
[[Category: Hallberg BM]]
[[Category: Hallberg, B M]]
[[Category: Hammarstrom M]]
[[Category: Hammarstrom, M]]
[[Category: Hogbom M]]
[[Category: Hogbom, M]]
[[Category: Holmberg-Schiavone L]]
[[Category: Holmberg-Schiavone, L]]
[[Category: Johansson I]]
[[Category: Johansson, I]]
[[Category: Karlberg T]]
[[Category: Karlberg, T]]
[[Category: Kotenyova T]]
[[Category: Kotenyova, T]]
[[Category: Moche M]]
[[Category: Moche, M]]
[[Category: Nilsson ME]]
[[Category: Nilsson, M E]]
[[Category: Nordlund P]]
[[Category: Nordlund, P]]
[[Category: Nyman T]]
[[Category: Nyman, T]]
[[Category: Ogg D]]
[[Category: Ogg, D]]
[[Category: Persson C]]
[[Category: Persson, C]]
[[Category: Sagemark J]]
[[Category: Structural genomic]]
[[Category: Stenmark P]]
[[Category: Sagemark, J]]
[[Category: Sundstrom M]]
[[Category: Stenmark, P]]
[[Category: Thorsell AG]]
[[Category: Sundstrom, M]]
[[Category: Uppenberg J]]
[[Category: Thorsell, A G]]
[[Category: Van Den Berg S]]
[[Category: Uppenberg, J]]
[[Category: Wallden K]]
[[Category: Wallden, K]]
[[Category: Weigelt J]]
[[Category: Weigelt, J]]
[[Category: Amino-acid biosynthesis]]
[[Category: Ligase]]
[[Category: Sgc]]
[[Category: Synthetase]]

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