1khb

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[[Image:1khb.jpg|left|200px]]
 
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==PEPCK complex with nonhydrolyzable GTP analog, native data==
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The line below this paragraph, containing "STRUCTURE_1khb", creates the "Structure Box" on the page.
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<StructureSection load='1khb' size='340' side='right'caption='[[1khb]], [[Resolution|resolution]] 1.85&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'>[[1khb]] 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=1KHB OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1KHB 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]] 1.854&#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=ACT:ACETATE+ION'>ACT</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=GCP:PHOSPHOMETHYLPHOSPHONIC+ACID+GUANYLATE+ESTER'>GCP</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene></td></tr>
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{{STRUCTURE_1khb| PDB=1khb | 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=1khb FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1khb OCA], [https://pdbe.org/1khb PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1khb RCSB], [https://www.ebi.ac.uk/pdbsum/1khb PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1khb ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PCKGC_HUMAN PCKGC_HUMAN] Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD) [MIM:[https://omim.org/entry/261680 261680]. A metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.
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== Function ==
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[https://www.uniprot.org/uniprot/PCKGC_HUMAN PCKGC_HUMAN] Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.
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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/kh/1khb_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=1khb ConSurf].
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<div style="clear:both"></div>
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'''PEPCK complex with nonhydrolyzable GTP analog, native data'''
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==See Also==
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*[[Phosphoenolpyruvate carboxykinase 3D structures|Phosphoenolpyruvate carboxykinase 3D structures]]
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__TOC__
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==Overview==
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</StructureSection>
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We report crystal structures of the human enzyme phosphoenolpyruvate carboxykinase (PEPCK) with and without bound substrates. These structures are the first to be determined for a GTP-dependent PEPCK, and provide the first view of a novel GTP-binding site unique to the GTP-dependent PEPCK family. Three phenylalanine residues form the walls of the guanine-binding pocket on the enzyme's surface and, most surprisingly, one of the phenylalanine side-chains contributes to the enzyme's specificity for GTP. PEPCK catalyzes the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle. Because the gluconeogenic pathway contributes to the fasting hyperglycemia of type II diabetes, inhibitors of PEPCK may be useful in the treatment of diabetes.
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==Disease==
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Known disease associated with this structure: Hypoglycemia due to PCK1 deficiency (1) OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=261680 261680]]
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==About this Structure==
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1KHB is a [[Single protein]] 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=1KHB OCA].
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==Reference==
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Crystal structure of human cytosolic phosphoenolpyruvate carboxykinase reveals a new GTP-binding site., Dunten P, Belunis C, Crowther R, Hollfelder K, Kammlott U, Levin W, Michel H, Ramsey GB, Swain A, Weber D, Wertheimer SJ, J Mol Biol. 2002 Feb 15;316(2):257-64. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/11851336 11851336]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Belunis, C.]]
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[[Category: Belunis C]]
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[[Category: Crowther, R.]]
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[[Category: Crowther R]]
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[[Category: Dunten, P.]]
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[[Category: Dunten P]]
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[[Category: Hollfelder, K.]]
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[[Category: Hollfelder K]]
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[[Category: Kammlott, U.]]
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[[Category: Kammlott U]]
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[[Category: Levin, W.]]
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[[Category: Levin W]]
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[[Category: Michel, H.]]
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[[Category: Michel H]]
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[[Category: Ramsey, G B.]]
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[[Category: Ramsey GB]]
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[[Category: Swain, A.]]
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[[Category: Swain A]]
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[[Category: Weber, D.]]
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[[Category: Weber D]]
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[[Category: Wertheimer, S J.]]
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[[Category: Wertheimer SJ]]
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[[Category: Gluconeogenesis]]
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[[Category: P-loop]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 22:44:48 2008''
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Current revision

PEPCK complex with nonhydrolyzable GTP analog, native data

PDB ID 1khb

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