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Gene Review

AGL  -  amylo-alpha-1, 6-glucosidase, 4-alpha...

Homo sapiens

Synonyms: GDE, Glycogen debrancher, Glycogen debranching enzyme
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Disease relevance of AGL


High impact information on AGL


Chemical compound and disease context of AGL


Biological context of AGL


Anatomical context of AGL

  • In this study, we found that a single injection of AGL administered within 2 hours of lethal irradiation resulted in the long-term survival of mice without bone marrow transplantation [14].
  • Peripheral blood hematology showed that AGL administration accelerated the recovery of hematopoietic parameters, including reticulocytes and red and white blood cells [14].
  • The high frequency of this syndrome during the course of AGL and of acute phase of CGL seems to be linked to the low deformability of the myeloblasts [3].
  • In the present report we investigated T cell responses to A-gliadin (AGL), a major alpha-gliadin component known to activate disease [4].
  • The 5 HT organelles/volume ratio was normal in AGL and ALL but was significantly decreased in AML [15].

Associations of AGL with chemical compounds

  • Thus it is likely that the AMPK-GDE association is a novel mechanism regulating AMPK activity and the resultant fatty acid oxidation and glucose uptake [16].
  • Mass spectrometry and a Mascot search revealed this protein to be a glycogen debranching enzyme (GDE) [16].
  • The quail glycoprotein order of affinities was: Con A >> WGA = AGL = PA-IlL, while that of the pigeon was: AGL > PA-IL > WGA > Con A = PA-IlL [17].
  • AGL itself displayed no colony-stimulating activity, but AGL-stimulated spleen cell-conditioned medium (AGL-SCM) promoted the proliferation and differentiation of bone marrow mononuclear cells from normal mice and Lin marrow cells from 5-fluorouracil (5-FU)-treated mice [14].
  • AGL 2592 induces a dose-dependent and time-dependent inhibition of tyrosine phosphorylation of numerous proteins, including Stat3, and an increase of Bcl-2 phosphorylation, both biochemical hallmarks of growth inhibition and apoptosis [18].

Regulatory relationships of AGL

  • These findings suggest that AGL may be a useful in treating radiation-induced hematopoietic damage [14].

Other interactions of AGL

  • The predominant form of human liver AGL cDNA (isoform 1) contained 400 bp of 5' untranslated region, 4596 bp of coding region, and 2371 bp of 3' untranslated region [12].
  • Herein, we describe the identification of a family of PTK inhibitors whose most potent member is AGL 2592 [18].
  • 1. We examined whether N-hydroxyethyl-1-deoxynojirimycin (miglitol), a new human anti-diabetic drug with effects to inhibit alpha-1, 6-glucosidase glycogen debranching enzyme and reduce the glycogenolytic rate as well as to inhibit alpha-1,4-glucosidase, could reduce infarct size in the rabbit heart [19].

Analytical, diagnostic and therapeutic context of AGL


  1. Human glycogen debranching enzyme gene (AGL): complete structural organization and characterization of the 5' flanking region. Bao, Y., Dawson, T.L., Chen, Y.T. Genomics (1996) [Pubmed]
  2. A nonsense mutation due to a single base insertion in the 3'-coding region of glycogen debranching enzyme gene associated with a severe phenotype in a patient with glycogen storage disease type IIIa. Shen, J., Bao, Y., Chen, Y.T. Hum. Mutat. (1997) [Pubmed]
  3. Respiratory distress of hyperleukocytic granulocytic leukemias. Vernant, J.P., Brun, B., Mannoni, P., Dreyfus, B. Cancer (1979) [Pubmed]
  4. Peripheral T cell response to A-gliadin in celiac disease: differential processing and presentation capacities of Epstein-Barr-transformed B cells and fibroblasts. Franco, A., Appella, E., Kagnoff, M.F., Chowers, Y., Sakaguchi, K., Grey, H.M., Sette, A. Clin. Immunol. Immunopathol. (1994) [Pubmed]
  5. Hepatic and neuromuscular forms of glycogenosis type III: nine mutations in AGL. Lucchiari, S., Pagliarani, S., Salani, S., Filocamo, M., Di Rocco, M., Melis, D., Rodolico, C., Musumeci, O., Toscano, A., Bresolin, N., Comi, G.P. Hum. Mutat. (2006) [Pubmed]
  6. 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. Shen, J., Bao, Y., Liu, H.M., Lee, P., Leonard, J.V., Chen, Y.T. J. Clin. Invest. (1996) [Pubmed]
  7. Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy. Coleman, R.A., Winter, H.S., Wolf, B., Gilchrist, J.M., Chen, Y.T. Ann. Intern. Med. (1992) [Pubmed]
  8. Molecular cloning and nucleotide sequence of cDNA encoding human muscle glycogen debranching enzyme. Yang, B.Z., Ding, J.H., Enghild, J.J., Bao, Y., Chen, Y.T. J. Biol. Chem. (1992) [Pubmed]
  9. Glycogen storage disease type III-hepatocellular carcinoma a long-term complication? Demo, E., Frush, D., Gottfried, M., Koepke, J., Boney, A., Bali, D., Chen, Y.T., Kishnani, P.S. J. Hepatol. (2007) [Pubmed]
  10. Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features. Coleman, R.A., Winter, H.S., Wolf, B., Chen, Y.T. J. Inherit. Metab. Dis. (1992) [Pubmed]
  11. Cloning and nucleotide sequence of a gene encoding a glycogen debranching enzyme in the trehalose operon from Arthrobacter sp. Q36. Maruta, K., Kubota, M., Fukuda, S., Kurimoto, M. Biochim. Biophys. Acta (2000) [Pubmed]
  12. Isolation and nucleotide sequence of human liver glycogen debranching enzyme mRNA: identification of multiple tissue-specific isoforms. Bao, Y., Yang, B.Z., Dawson, T.L., Chen, Y.T. Gene (1997) [Pubmed]
  13. Mutational and haplotype analysis of AGL in patients with glycogen storage disease type III. Horinishi, A., Okubo, M., Tang, N.L., Hui, J., To, K.F., Mabuchi, T., Okada, T., Mabuchi, H., Murase, T. J. Hum. Genet. (2002) [Pubmed]
  14. alpha-Galactosylceramide (AGL-517) treatment protects mice from lethal irradiation. Inoue, H., Koezuka, Y., Nishi, N., Osawa, M., Motoki, K., Kobayashi, E., Kabaya, K., Obuchi, M., Fukushima, H., Mori, K.J. Exp. Hematol. (1997) [Pubmed]
  15. Platelet volume, density and 5 HT organelles (mepacrine test) in acute leukaemia. Nouvel, C., Caranobe, C., Sie, P., Capdeville, J., Pris, J., Boneu, B. Scandinavian journal of haematology. (1978) [Pubmed]
  16. Glycogen debranching enzyme association with beta-subunit regulates AMP-activated protein kinase activity. Sakoda, H., Fujishiro, M., Fujio, J., Shojima, N., Ogihara, T., Kushiyama, A., Fukushima, Y., Anai, M., Ono, H., Kikuchi, M., Horike, N., Viana, A.Y., Uchijima, Y., Kurihara, H., Asano, T. Am. J. Physiol. Endocrinol. Metab. (2005) [Pubmed]
  17. Differential staining of western blots of avian egg white glycoproteins using diverse lectins. Lerrer, B., Gilboa-Garber, N. Electrophoresis (2002) [Pubmed]
  18. Tyrosine kinase inhibitors suppress the growth of non-hodgkin B lymphomas. Ben-Bassat, H., Hartzstark, Z., Levitzki, R., Klein, B.Y., Shlomai, Z., Gazit, A., Levitzki, A. J. Pharmacol. Exp. Ther. (2002) [Pubmed]
  19. A novel anti-diabetic drug, miglitol, markedly reduces myocardial infarct size in rabbits. Minatoguchi, S., Arai, M., Uno, Y., Kariya, T., Nishida, Y., Hashimoto, K., Kawasaki, M., Takemura, G., Fujiwara, T., Fujiwara, H. Br. J. Pharmacol. (1999) [Pubmed]
  20. Molecular genetic basis and prevalence of glycogen storage disease type IIIA in the Faroe Islands. Santer, R., Kinner, M., Steuerwald, U., Kjaergaard, S., Skovby, F., Simonsen, H., Shaiu, W.L., Chen, Y.T., Schneppenheim, R., Schaub, J. Eur. J. Hum. Genet. (2001) [Pubmed]
  21. Prenatal diagnosis and carrier detection for glycogen storage disease type III using polymorphic DNA markers. Shen, J., Liu, H.M., McConkie-Rosell, A., Chen, Y.T. Prenat. Diagn. (1998) [Pubmed]
  22. Novel lectins from rhizomes of two Acorus species with mitogenic activity and inhibitory potential towards murine cancer cell lines. Bains, J.S., Dhuna, V., Singh, J., Kamboj, S.S., Nijjar, K.K., Agrewala, J.N. Int. Immunopharmacol. (2005) [Pubmed]
  23. Blood ammonia--heart rate relationship during graded exercise is not influenced by glycogen depletion. Roeykens, J., Magnus, L., Rogers, R., Meeusen, R., De Meirleir, K. International journal of sports medicine. (1998) [Pubmed]
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