Gene Symbol: Agl
Description: amylo-1,6-glucosidase, 4-alpha-glucanotransferase
Alias: 1110061O17Rik, 9430004C13Rik, 9630046L06Rik, AI850929, C77197, glycogen debranching enzyme
Sakoda H, Fujishiro M, Fujio J, Shojima N, Ogihara T, Kushiyama A, et al
. Glycogen debranching enzyme association with beta-subunit regulates AMP-activated protein kinase activity. Am J Physiol Endocrinol Metab. 2005;289:E474-81 pubmed
..Mass spectrometry and a Mascot search revealed this protein to be a glycogen debranching enzyme (GDE)...
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)...
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. ..
Probert F, Rice P, Scudamore C, Wells S, Williams R, Hough T, et al
. Â¹H NMR metabolic profiling of plasma reveals additional phenotypes in knockout mouse models. J Proteome Res. 2015;14:2036-45 pubmed publisher
..Significant differences were observed in two KO lines, Agl (MGI: 1924809) and Bbs5 (MGI: 1919819), by (1)H NMR spectroscopy, clinical chemistry, and histopathology...
Liu S, Li Y, Chen Y, Chiang E, Li A, Lee Y, et al
. Glycine N-methyltransferase-/- mice develop chronic hepatitis and glycogen storage disease in the liver. Hepatology. 2007;46:1413-25 pubmed
Liu K, Wu J, Chen Y. Mouse model of glycogen storage disease type III. Mol Genet Metab. 2014;111:467-76 pubmed publisher
Glycogen storage disease type IIIa (GSD IIIa) is caused by a deficiency of the glycogen debranching enzyme (GDE), which is encoded by the Agl gene...
Pagliarani S, Lucchiari S, Ulzi G, Violano R, Ripolone M, Bordoni A, et al
. Glycogen storage disease type III: A novel Agl knockout mouse model. Biochim Biophys Acta. 2014;1842:2318-28 pubmed publisher
Glycogen storage disease type III is an autosomal recessive disease characterized by a deficiency in the glycogen debranching enzyme, encoded by AGL...