3ceh

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[[Image:3ceh.png|left|200px]]
 
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{{STRUCTURE_3ceh| PDB=3ceh | SCENE= }}
{{STRUCTURE_3ceh| PDB=3ceh | SCENE= }}
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===Human liver glycogen phosphorylase (tense state) in complex with the allosteric inhibitor AVE5688===
===Human liver glycogen phosphorylase (tense state) in complex with the allosteric inhibitor AVE5688===
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{{ABSTRACT_PUBMED_18373353}}
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{{ABSTRACT_PUBMED_18373353}}
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==Disease==
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[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:[http://omim.org/entry/232700 232700]]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.<ref>PMID:9529348</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties.
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:018373353</ref><references group="xtra"/>
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<ref group="xtra">PMID:018373353</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Phosphorylase]]
[[Category: Phosphorylase]]

Revision as of 09:11, 24 March 2013

Template:STRUCTURE 3ceh

Contents

Human liver glycogen phosphorylase (tense state) in complex with the allosteric inhibitor AVE5688

Template:ABSTRACT PUBMED 18373353

Disease

[PYGL_HUMAN] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:232700]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.[1]

Function

[PYGL_HUMAN] Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties.

About this Structure

3ceh is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Anderka O, Loenze P, Klabunde T, Dreyer MK, Defossa E, Wendt KU, Schmoll D. Thermodynamic characterization of allosteric glycogen phosphorylase inhibitors. Biochemistry. 2008 Apr 22;47(16):4683-91. Epub 2008 Mar 29. PMID:18373353 doi:10.1021/bi702397d
  1. ↑ Burwinkel B, Bakker HD, Herschkovitz E, Moses SW, Shin YS, Kilimann MW. Mutations in the liver glycogen phosphorylase gene (PYGL) underlying glycogenosis type VI. Am J Hum Genet. 1998 Apr;62(4):785-91. PMID:9529348

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