2ati

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[[Image:2ati.png|left|200px]]
 
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{{STRUCTURE_2ati| PDB=2ati | SCENE= }}
{{STRUCTURE_2ati| PDB=2ati | SCENE= }}
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===Glycogen Phosphorylase Inhibitors===
===Glycogen Phosphorylase Inhibitors===
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{{ABSTRACT_PUBMED_16190745}}
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{{ABSTRACT_PUBMED_16190745}}
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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:016190745</ref><ref group="xtra">PMID:015987904</ref><ref group="xtra">PMID:010949035</ref><references group="xtra"/>
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<ref group="xtra">PMID:016190745</ref><ref group="xtra">PMID:015987904</ref><ref group="xtra">PMID:010949035</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Phosphorylase]]
[[Category: Phosphorylase]]

Revision as of 22:21, 24 March 2013

Template:STRUCTURE 2ati

Contents

Glycogen Phosphorylase Inhibitors

Template:ABSTRACT PUBMED 16190745

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

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

See Also

Reference

  • Klabunde T, Wendt KU, Kadereit D, Brachvogel V, Burger HJ, Herling AW, Oikonomakos NG, Kosmopoulou MN, Schmoll D, Sarubbi E, von Roedern E, Schonafinger K, Defossa E. Acyl ureas as human liver glycogen phosphorylase inhibitors for the treatment of type 2 diabetes. J Med Chem. 2005 Oct 6;48(20):6178-93. PMID:16190745 doi:10.1021/jm049034y
  • Oikonomakos NG, Kosmopoulou MN, Chrysina ED, Leonidas DD, Kostas ID, Wendt KU, Klabunde T, Defossa E. Crystallographic studies on acyl ureas, a new class of glycogen phosphorylase inhibitors, as potential antidiabetic drugs. Protein Sci. 2005 Jul;14(7):1760-71. PMID:15987904 doi:14/7/1760
  • Rath VL, Ammirati M, LeMotte PK, Fennell KF, Mansour MN, Danley DE, Hynes TR, Schulte GK, Wasilko DJ, Pandit J. Activation of human liver glycogen phosphorylase by alteration of the secondary structure and packing of the catalytic core. Mol Cell. 2000 Jul;6(1):139-48. PMID:10949035
  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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