1khg

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[[Image:1khg.png|left|200px]]
 
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{{STRUCTURE_1khg| PDB=1khg | SCENE= }}
{{STRUCTURE_1khg| PDB=1khg | SCENE= }}
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===PEPCK===
===PEPCK===
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{{ABSTRACT_PUBMED_11851336}}
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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.
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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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==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:011851336</ref><references group="xtra"/>
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<ref group="xtra">PMID:011851336</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Belunis, C.]]
[[Category: Belunis, C.]]

Revision as of 20:46, 24 March 2013

Template:STRUCTURE 1khg

Contents

PEPCK

Template:ABSTRACT PUBMED 11851336

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

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

Reference

  • 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

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OCA

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