Glycogen debranching

FIGURE 23.15 The reactions of glycogen debranching enzyme. Transfer of a group of three o -(l 4)-linked glucose residues from a limit branch to another branch is followed by cleavage of the o -(l 6) bond of the residue... 

Tabata, S. and Dohi, Y., An assay for oligo-(l—>4) —5(1—>4)-glucantransferase activity in the glycogen debranching enzyme system by using HPLC with a pulsed amperometric detector, Carb. Res., 230,179, 1992. 

III Cori Glycogen debranching enzyme Mild hypoglycemia, liver enlargement Short outer branches Single glucose residue at outer branch... 

GLYCINE REDUCTASE GLYCINE SYNTHASE Glycogen debranching enzyme, DEBRANCHING ENZYME Glycogenolysis tracer kinetics,... 

Brown DH, Brown BI (1966) Enzymes of glycogen debranching amylo-l,6-glucosidase and oligo-l,4->l,4-glucantransferase. Methods Enzymol 8 515-524... 

Chen YT, He JK, Ding JH, Brown BI (1987) Glycogen debranching enzyme purification, antibody characterization, and immunoblot analysis of type III glycogen storage disease. Am J Hum Genet 41 1002-1015... 

Glycogen degradation requires two enzymes glycogen phosphorylase and glycogen-debranching enzyme. 

The Coordinated Action of the Two Glycogen Debranching Enzyme Activities on Phosphorylase Limit Dextrin... 

The glycogen debranching enzyme is the first bifunctional eukaryotic enzyme to be reported that consists of a single polypeptide chain. It catalyzes two distinct activities an oligosaccharide frons-glycosylation followed by hydrolysis of an (1—> 6)-linked D-glucosyl unit to liberate free glucose. Physical-chemical and kinetic characterization of this novel bifunctional enzyme is described by Nelson. 

Jespersen, H. M., MacGregor, A., Henrissat, B., Sierks, M. R., and Svensson, B. 1993. Starch-and glycogen-debranching and branching enzymes prediction of structural features of the catalytic (/S/a)g-barrel domain and evolutionary relationship to other amylolytic enzymes. J. Protein Chem. 12,781-805. 

III Glycogen debrancher Cori s disease or Forbe s disease Enlarged liver fasting hypoglycaemia myopathy... 

Glycogen branching requires a single transferase activity. Glycogen debranching requires two enzyme activities a transferase and an a-1,6 glucosidase. Sequence analysis suggests that the two transferases and, perhaps, the a-1,6... 

Family resemblance. Propose mechanisms for the two enzymes catalyzing steps in glycogen debranching based on their potential membership in the a-amylase family. 

Fitzgerald, P. M. D., and Madsen, N. B. J. 1986. Improvement of limit of diffraction and useful X-ray lifetime of crystals of glycogen debranching enzyme. J. Cryst. Growth 76 600-606. 

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