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systém enzýmový, rušiaci vetvenie glykogénu

  1. Subject h.systém enzýmový, rušiaci vetvenie glykogénu
    Subject h.Glycogen Debranching Enzyme System
    English X referencesGlycogen Debranching Enzyme
    Transfer-Glucosidase
    Scope note in English1,4-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.
    See also reference (FX) in Slovak glykogenóza, typ III
    See also reference (FX) in English Glycogen Storage Disease Type III
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