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1m51

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[[Image:1m51.png|left|200px]]
 
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{{STRUCTURE_1m51| PDB=1m51 | SCENE= }}
{{STRUCTURE_1m51| PDB=1m51 | SCENE= }}
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===PEPCK complex with a GTP-competitive inhibitor===
===PEPCK complex with a GTP-competitive inhibitor===
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{{ABSTRACT_PUBMED_14552798}}
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{{ABSTRACT_PUBMED_14552798}}
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==Disease==
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[[http://www.uniprot.org/uniprot/PCKGC_HUMAN PCKGC_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.
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==Function==
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[[http://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.
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:014552798</ref><references group="xtra"/>
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<ref group="xtra">PMID:014552798</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Dunten, P.]]
[[Category: Dunten, P.]]

Revision as of 17:26, 24 March 2013

Template:STRUCTURE 1m51

Contents

PEPCK complex with a GTP-competitive inhibitor

Template:ABSTRACT PUBMED 14552798

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.

About this Structure

1m51 is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • 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

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OCA

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