1aos

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{{STRUCTURE_1aos| PDB=1aos | SCENE= }}
 
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===HUMAN ARGININOSUCCINATE LYASE===
 
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{{ABSTRACT_PUBMED_9256435}}
 
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==Disease==
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==HUMAN ARGININOSUCCINATE LYASE==
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[[http://www.uniprot.org/uniprot/ARLY_HUMAN ARLY_HUMAN]] Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA) [MIM:[http://omim.org/entry/207900 207900]]. An autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness.<ref>PMID:1705937</ref><ref>PMID:2263616</ref><ref>PMID:12408190</ref><ref>PMID:17326097</ref>
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<StructureSection load='1aos' size='340' side='right'caption='[[1aos]], [[Resolution|resolution]] 4.20&Aring;' scene=''>
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== Structural highlights ==
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==About this Structure==
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<table><tr><td colspan='2'>[[1aos]] is a 2 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=1AOS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1AOS FirstGlance]. <br>
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[[1aos]] is a 2 chain structure with 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=1AOS OCA].
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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]] 4.2&#8491;</td></tr>
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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=1aos FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1aos OCA], [https://pdbe.org/1aos PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1aos RCSB], [https://www.ebi.ac.uk/pdbsum/1aos PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1aos ProSAT]</span></td></tr>
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==Reference==
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</table>
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<ref group="xtra">PMID:009256435</ref><references group="xtra"/><references/>
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== Disease ==
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[[Category: Argininosuccinate lyase]]
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[https://www.uniprot.org/uniprot/ARLY_HUMAN ARLY_HUMAN] Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA) [MIM:[https://omim.org/entry/207900 207900]. An autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness.<ref>PMID:1705937</ref> <ref>PMID:2263616</ref> <ref>PMID:12408190</ref> <ref>PMID:17326097</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ARLY_HUMAN ARLY_HUMAN]
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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/ao/1aos_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=1aos ConSurf].
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<div style="clear:both"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Howell, P L.]]
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[[Category: Large Structures]]
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[[Category: Mcinnes, R R.]]
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[[Category: Howell PL]]
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[[Category: Simpson, A.]]
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[[Category: Mcinnes RR]]
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[[Category: Turner, M A.]]
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[[Category: Simpson A]]
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[[Category: Arginine biosynthesis]]
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[[Category: Turner MA]]
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[[Category: Lyase]]
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[[Category: Urea cycle]]
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Current revision

HUMAN ARGININOSUCCINATE LYASE

PDB ID 1aos

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