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[[Image:2nz2.gif|left|200px]]<br /><applet load="2nz2" size="350" color="white" frame="true" align="right" spinBox="true"
caption="2nz2, resolution 2.40&Aring;" />
'''Crystal structure of human argininosuccinate synthase in complex with aspartate and citrulline'''<br />


==Disease==
==Crystal structure of human argininosuccinate synthase in complex with aspartate and citrulline==
Known disease associated with this structure: Citrullinemia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=603470 603470]]
<StructureSection load='2nz2' size='340' side='right'caption='[[2nz2]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[2nz2]] 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=2NZ2 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2NZ2 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]] 2.4&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ASP:ASPARTIC+ACID'>ASP</scene>, <scene name='pdbligand=CIR:CITRULLINE'>CIR</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</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=2nz2 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2nz2 OCA], [https://pdbe.org/2nz2 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2nz2 RCSB], [https://www.ebi.ac.uk/pdbsum/2nz2 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2nz2 ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ASSY_HUMAN ASSY_HUMAN] Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1) [MIM:[https://omim.org/entry/215700 215700]. Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood.<ref>PMID:11941481</ref> <ref>PMID:2358466</ref> <ref>PMID:1943692</ref> <ref>PMID:7977368</ref> <ref>PMID:8792870</ref> <ref>PMID:11708871</ref> <ref>PMID:12815590</ref> <ref>PMID:14680976</ref> <ref>PMID:16475226</ref>
== Function ==
[https://www.uniprot.org/uniprot/ASSY_HUMAN ASSY_HUMAN]
== 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/nz/2nz2_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=2nz2 ConSurf].
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Argininosuccinate synthetase catalyzes the transformation of citrulline and aspartate into argininosuccinate and pyrophosphate using the hydrolysis of ATP to AMP and pyrophosphate. This enzymatic process constitutes the rate-limiting step in both the urea and arginine-citrulline cycles. Previous studies have investigated the crystal structures of argininosuccinate synthetase from bacterial species. In this work, the first crystal structure of human argininosuccinate synthetase in complex with the substrates citrulline and aspartate is presented. The human enzyme is compared with structures of argininosuccinate synthetase from bacteria. In addition, the structure also provides new insights into the function of the numerous clinical mutations identified in patients with type I citrullinaemia (also known as classic citrullinaemia).


==About this Structure==
Structure of human argininosuccinate synthetase.,Karlberg T, Collins R, van den Berg S, Flores A, Hammarstrom M, Hogbom M, Holmberg Schiavone L, Uppenberg J Acta Crystallogr D Biol Crystallogr. 2008 Mar;64(Pt 3):279-86. Epub 2008, Feb 20. PMID:18323623<ref>PMID:18323623</ref>
2NZ2 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=NA:'>NA</scene>, <scene name='pdbligand=ASP:'>ASP</scene> and <scene name='pdbligand=CIR:'>CIR</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Argininosuccinate_synthase Argininosuccinate synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=6.3.4.5 6.3.4.5] Known structural/functional Sites: <scene name='pdbsite=AC1:ASP+Binding+Site+For+Residue+A+501'>AC1</scene> and <scene name='pdbsite=AC2:Cir+Binding+Site+For+Residue+A+502'>AC2</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2NZ2 OCA].
 
[[Category: Argininosuccinate synthase]]
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 2nz2" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[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: Ericsson UB]]
[[Category: Ericsson, U B.]]
[[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: Magnusdottir A]]
[[Category: Magnusdottir, A.]]
[[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: SGC, Structural Genomics Consortium.]]
[[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: ASP]]
[[Category: CIR]]
[[Category: NA]]
[[Category: amino-acid biosynthesis]]
[[Category: aspartate]]
[[Category: citrulline]]
[[Category: ligase]]
[[Category: sgc]]
[[Category: structural genomics]]
[[Category: structural genomics consortium]]
[[Category: synthase]]
 
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