Gene Symbol: Agl
Description: amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
Alias: glycogen debranching enzyme, amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III), amylo-1,6-glucosidase, 4-alpha-glucanotransferase
Species: rat
Products:     Agl

Top Publications

  1. Nishida Y, Minatoguchi S, Arai M, Takemura G, Uno Y, Hashimoto K, et al. N-methyl-1-deoxynojirimycin (MOR-14), an alpha-glucosidase inhibitor, markedly improves postischemic left ventricular dysfunction. Heart Vessels. 2000;15:268-73 pubmed
    ..Preischemic treatment with MOR-14 is protective against postischemic left ventricular dysfunction through the inhibition of glycogenolysis in the isolated rat heart. ..
  2. Lees S, Chen Y, Williams J. Glycogen debranching enzyme is associated with rat skeletal muscle sarcoplasmic reticulum. Acta Physiol Scand. 2004;181:239-45 pubmed
    ..This investigation sought to examine glycogen debranching enzyme associated with skeletal muscle SR...
  3. Vaillant R, Bourgeois Victor P, Fairand A. [Development and control of liver amylo-1,6-glucosidase activity in the fetal rat]. Can J Biochem. 1979;57:1245-9 pubmed
  4. Mannan A, Noujaim A, Wiebe L, Secord D, Silverberg D. Carbohydrate metabolism in the chronically uremic rat. Clin Biochem. 1975;8:194-8 pubmed
    ..Changes in phosphorylase "a" to "b" activity ratios and synthetase I to D activity ratios were found in the uremics. The biochemical significance of these alterations are discussed. ..
  5. Popova I, Presnova V, Lavrenova T. [Effect of biogenic amines on glycogen degradation in isolated rat hepatocytes]. Vopr Med Khim. 1992;38:30-3 pubmed
    ..Tyramine may be used as stimulator of glycogen accumulation in liver cells. ..
  6. Chen Y, He J, Ding J, Brown B. Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease. Am J Hum Genet. 1987;41:1002-15 pubmed
  7. Depre C, Hue L. Inhibition of glycogenolysis by a glucose analogue in the working rat heart. J Mol Cell Cardiol. 1997;29:2253-9 pubmed
  8. Schliselfeld L, Danon M. Inverse relationship of skeletal muscle glycogen from wild-type and genetically modified mice to their phosphorylase a activity. Biochem Biophys Res Commun. 2002;290:874-7 pubmed
    ..00 mM AMP (4.8% and less glycogen phosphorylase a). This suggests that there is an inverse relationship between mouse muscle phosphorylase a and the muscle's glycogen content. ..
  9. Lucchiari S, Pagliarani S, Salani S, Filocamo M, Di Rocco M, Melis D, et al. Hepatic and neuromuscular forms of glycogenosis type III: nine mutations in AGL. Hum Mutat. 2006;27:600-1 pubmed
    Glycogenosis type III (Cori disease) is an autosomal recessive disorder caused by the deficiency of the glycogen debranching enzyme, encoded by the AGL gene, and existing in six isoforms alternately spliced in a tissue-specific way...

More Information


  1. Cheng A, Zhang M, Gentry M, Worby C, Dixon J, Saltiel A. A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease. Genes Dev. 2007;21:2399-409 pubmed
    Cori's disease is a glycogen storage disorder characterized by a deficiency in the glycogen debranching enzyme, amylo-1,6-glucosidase,4-alpha-glucanotransferase (AGL)...
  2. Satoh K, Sato K. Glycogen-binding protein components of rat tissues. Biochem Biophys Res Commun. 1980;96:28-33 pubmed
  3. Shen J, Bao Y, Liu H, Lee P, Leonard J, Chen Y. Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle. J Clin Invest. 1996;98:352-7 pubmed
    Glycogen storage disease type HI (GSD-III), an autosomal recessive disease, is caused by deficient glycogen debranching enzyme (GDE) activity...