2gmv

From Proteopedia

(Difference between revisions)
Jump to: navigation, search
Line 1: Line 1:
-
[[Image:2gmv.png|left|200px]]
 
- 
{{STRUCTURE_2gmv| PDB=2gmv | SCENE= }}
{{STRUCTURE_2gmv| PDB=2gmv | SCENE= }}
- 
===PEPCK complex with a GTP-competitive inhibitor===
===PEPCK complex with a GTP-competitive inhibitor===
 +
{{ABSTRACT_PUBMED_17532214}}
-
{{ABSTRACT_PUBMED_17532214}}
+
==Disease==
 +
[[http://www.uniprot.org/uniprot/PPCKC_HUMAN PPCKC_HUMAN]] Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD) [MIM:[http://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==
 +
[[http://www.uniprot.org/uniprot/PPCKC_HUMAN PPCKC_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.
==About this Structure==
==About this Structure==
Line 11: Line 13:
==Reference==
==Reference==
-
<ref group="xtra">PMID:017532214</ref><ref group="xtra">PMID:014552798</ref><ref group="xtra">PMID:011851336</ref><references group="xtra"/>
+
<ref group="xtra">PMID:017532214</ref><ref group="xtra">PMID:014552798</ref><ref group="xtra">PMID:011851336</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Dunten, P.]]
[[Category: Dunten, P.]]

Revision as of 17:47, 24 March 2013

Template:STRUCTURE 2gmv

Contents

PEPCK complex with a GTP-competitive inhibitor

Template:ABSTRACT PUBMED 17532214

Disease

[PPCKC_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

[PPCKC_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.

About this Structure

2gmv is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Pietranico SL, Foley LH, Huby N, Yun W, Dunten P, Vermeulen J, Wang P, Toth K, Ramsey G, Gubler ML, Wertheimer SJ. C-8 Modifications of 3-alkyl-1,8-dibenzylxanthines as inhibitors of human cytosolic phosphoenolpyruvate carboxykinase. Bioorg Med Chem Lett. 2007 Jul 15;17(14):3835-9. Epub 2007 May 22. PMID:17532214 doi:http://dx.doi.org/10.1016/j.bmcl.2007.05.013
  • Foley LH, Wang P, Dunten P, Ramsey G, Gubler ML, Wertheimer SJ. X-ray structures of two xanthine inhibitors bound to PEPCK and N-3 modifications of substituted 1,8-dibenzylxanthines. Bioorg Med Chem Lett. 2003 Nov 3;13(21):3871-4. PMID:14552798
  • Dunten P, Belunis C, Crowther R, Hollfelder K, Kammlott U, Levin W, Michel H, Ramsey GB, Swain A, Weber D, Wertheimer SJ. Crystal structure of human cytosolic phosphoenolpyruvate carboxykinase reveals a new GTP-binding site. J Mol Biol. 2002 Feb 15;316(2):257-64. PMID:11851336 doi:http://dx.doi.org/10.1006/jmbi.2001.5364

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/2gmv"
Personal tools