1khe
From Proteopedia
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==PEPCK complex with nonhydrolyzable GTP analog, MAD data== | ==PEPCK complex with nonhydrolyzable GTP analog, MAD data== | ||
| - | <StructureSection load='1khe' size='340' side='right' caption='[[1khe]], [[Resolution|resolution]] 2.40Å' scene=''> | + | <StructureSection load='1khe' size='340' side='right'caption='[[1khe]], [[Resolution|resolution]] 2.40Å' scene=''> |
== Structural highlights == | == Structural highlights == | ||
| - | <table><tr><td colspan='2'>[[1khe]] is a 1 chain structure with sequence from [ | + | <table><tr><td colspan='2'>[[1khe]] 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=1KHE OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1KHE FirstGlance]. <br> |
| - | </td></tr><tr id=' | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.4Å</td></tr> |
| - | <tr id=' | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GCP:PHOSPHOMETHYLPHOSPHONIC+ACID+GUANYLATE+ESTER'>GCP</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</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=1khe FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1khe OCA], [https://pdbe.org/1khe PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1khe RCSB], [https://www.ebi.ac.uk/pdbsum/1khe PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1khe ProSAT]</span></td></tr> | |
| - | + | ||
| - | < | + | |
| - | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[ | + | |
</table> | </table> | ||
== Disease == | == Disease == | ||
| - | [ | + | [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. |
== Function == | == Function == | ||
| - | [ | + | [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. |
== 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/kh/1khe_consurf.spt"</scriptWhenChecked> | + | <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/kh/1khe_consurf.spt"</scriptWhenChecked> |
| - | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/ | + | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked> |
<text>to colour the structure by Evolutionary Conservation</text> | <text>to colour the structure by Evolutionary Conservation</text> | ||
</jmolCheckbox> | </jmolCheckbox> | ||
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</div> | </div> | ||
<div class="pdbe-citations 1khe" style="background-color:#fffaf0;"></div> | <div class="pdbe-citations 1khe" style="background-color:#fffaf0;"></div> | ||
| + | |||
| + | ==See Also== | ||
| + | *[[Phosphoenolpyruvate carboxykinase 3D structures|Phosphoenolpyruvate carboxykinase 3D structures]] | ||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
| - | [[Category: | + | [[Category: Homo sapiens]] |
| - | [[Category: Belunis | + | [[Category: Large Structures]] |
| - | [[Category: Crowther | + | [[Category: Belunis C]] |
| - | [[Category: Dunten | + | [[Category: Crowther R]] |
| - | [[Category: Hollfelder | + | [[Category: Dunten P]] |
| - | [[Category: Kammlott | + | [[Category: Hollfelder K]] |
| - | [[Category: Levin | + | [[Category: Kammlott U]] |
| - | [[Category: Michel | + | [[Category: Levin W]] |
| - | [[Category: Ramsey | + | [[Category: Michel H]] |
| - | [[Category: Swain | + | [[Category: Ramsey GB]] |
| - | [[Category: Weber | + | [[Category: Swain A]] |
| - | [[Category: Wertheimer | + | [[Category: Weber D]] |
| - | + | [[Category: Wertheimer SJ]] | |
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Current revision
PEPCK complex with nonhydrolyzable GTP analog, MAD data
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Categories: Homo sapiens | Large Structures | Belunis C | Crowther R | Dunten P | Hollfelder K | Kammlott U | Levin W | Michel H | Ramsey GB | Swain A | Weber D | Wertheimer SJ
