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[[Image:1khb.jpg|left|200px]]


{{Structure
==PEPCK complex with nonhydrolyzable GTP analog, native data==
|PDB= 1khb |SIZE=350|CAPTION= <scene name='initialview01'>1khb</scene>, resolution 1.854&Aring;
<StructureSection load='1khb' size='340' side='right'caption='[[1khb]], [[Resolution|resolution]] 1.85&Aring;' scene=''>
|SITE=  
== Structural highlights ==
|LIGAND= <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>
<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>
|ACTIVITY= <span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphoenolpyruvate_carboxykinase_(GTP) Phosphoenolpyruvate carboxykinase (GTP)], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.1.1.32 4.1.1.32] </span>
</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>
|GENE= PCK1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
<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>
|DOMAIN=
<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>
|RELATEDENTRY=[[1khe|1KHE]], [[1khf|1KHF]], [[1khg|1KHG]]
</table>
|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1khb FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1khb OCA], [http://www.ebi.ac.uk/pdbsum/1khb PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1khb RCSB]</span>
== 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 ==
[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 ==
[[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/kh/1khb_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=1khb ConSurf].
<div style="clear:both"></div>


'''PEPCK complex with nonhydrolyzable GTP analog, native data'''
==See Also==
 
*[[Phosphoenolpyruvate carboxykinase 3D structures|Phosphoenolpyruvate carboxykinase 3D structures]]
 
__TOC__
==Overview==
</StructureSection>
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.
 
==Disease==
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]]
 
==About this Structure==
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].
 
==Reference==
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]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Phosphoenolpyruvate carboxykinase (GTP)]]
[[Category: Large Structures]]
[[Category: Single protein]]
[[Category: Belunis C]]
[[Category: Belunis, C.]]
[[Category: Crowther R]]
[[Category: Crowther, R.]]
[[Category: Dunten P]]
[[Category: Dunten, P.]]
[[Category: Hollfelder K]]
[[Category: Hollfelder, K.]]
[[Category: Kammlott U]]
[[Category: Kammlott, U.]]
[[Category: Levin W]]
[[Category: Levin, W.]]
[[Category: Michel H]]
[[Category: Michel, H.]]
[[Category: Ramsey GB]]
[[Category: Ramsey, G B.]]
[[Category: Swain A]]
[[Category: Swain, A.]]
[[Category: Weber D]]
[[Category: Weber, D.]]
[[Category: Wertheimer SJ]]
[[Category: Wertheimer, S J.]]
[[Category: gluconeogenesis]]
[[Category: p-loop]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 21:48:37 2008''

Latest revision as of 10:24, 14 February 2024

PEPCK complex with nonhydrolyzable GTP analog, native dataPEPCK complex with nonhydrolyzable GTP analog, native data

Structural highlights

1khb is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:X-ray diffraction, Resolution 1.854Å
Ligands:, , ,
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

PCKGC_HUMAN Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD) [MIM: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

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

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

See Also

1khb, resolution 1.85Å

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