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Glycogen Debranching Enzyme System

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Definition: 1,4-alpha-D-Glucan-1,4-alpha-D-glucan 4-alpha-D-glucosyltransferase/dextrin 6 alpha-D-glucanohydrolase. An enzyme system having both 4-alpha-glucanotransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) activities. As a transferase it transfers a segment of a 1,4-alpha-D-glucan to a new 4-position in an acceptor, which may be glucose or another 1,4-alpha-D-glucan. As a glucosidase it catalyzes the endohydrolysis of 1,6-alpha-D-glucoside linkages at points of branching in chains of 1,4-linked alpha-D-glucose residues. Amylo-1,6-glucosidase activity is deficient in glycogen storage disease type III.  /defic: consider also GLYCOGEN STORAGE DISEASE TYPE III   
See Also Glycogen Storage Disease Type III
Other names Transfer-Glucosidase; Glycogen Debranching Enzyme; Transfer Glucosidase; Enzyme, Glycogen Debranching; Debranching Enzyme, Glycogen
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
Igd1 protein, S cerevisiae  0   *Saccharomyces cerevisiae Proteins Glycogen Debranching Enzyme System/antagonists & inhibitors. FEMS Yeast Res. 2011 Sep 11;11(6):499-508.
amylo-1,6-glucosidase  EC 3.2.1.33   *Glycogen Debranching Enzyme System. Pediatrics 1981;67(1):107
4 alpha-glucanotransferase  EC 2.4.1.25   *Glycogen Debranching Enzyme System. Biochim Biophys Acta 566(1):100;1979

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012

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