1khb

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{{Seed}}
 
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[[Image:1khb.png|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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</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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</StructureSection>
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The line below this paragraph, {{ABSTRACT_PUBMED_11851336}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 11851336 is the PubMed ID number.
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{{ABSTRACT_PUBMED_11851336}}
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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 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=1KHB OCA].
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==Reference==
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<ref group="xtra">PMID:11851336</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Belunis, C.]]
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[[Category: Large Structures]]
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[[Category: Crowther, R.]]
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[[Category: Belunis C]]
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[[Category: Dunten, P.]]
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[[Category: Crowther R]]
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[[Category: Hollfelder, K.]]
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[[Category: Dunten P]]
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[[Category: Kammlott, U.]]
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[[Category: Hollfelder K]]
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[[Category: Levin, W.]]
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[[Category: Kammlott U]]
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[[Category: Michel, H.]]
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[[Category: Levin W]]
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[[Category: Ramsey, G B.]]
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[[Category: Michel H]]
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[[Category: Swain, A.]]
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[[Category: Ramsey GB]]
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[[Category: Weber, D.]]
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[[Category: Swain A]]
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[[Category: Wertheimer, S J.]]
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[[Category: Weber D]]
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[[Category: Gluconeogenesis]]
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[[Category: Wertheimer SJ]]
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[[Category: P-loop]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Feb 18 06:45:07 2009''
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Current revision

PEPCK complex with nonhydrolyzable GTP analog, native data

PDB ID 1khb

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