2qll

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Template:STRUCTURE 2qll

Contents

Human liver glycogen phosphorylase- GL complex

Template:ABSTRACT PUBMED 18198182

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

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

See Also

Reference

  • Pautsch A, Stadler N, Wissdorf O, Langkopf E, Moreth W, Streicher R. Molecular recognition of the protein phosphatase 1 glycogen targeting subunit by glycogen phosphorylase. J Biol Chem. 2008 Apr 4;283(14):8913-8. Epub 2008 Jan 15. PMID:18198182 doi:10.1074/jbc.M706612200
  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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