GLYCOGEN DEBRANCHING ENZYME
\ɡlˈa͡ɪkəd͡ʒən dˈɛbɹant͡ʃɪŋ ˈɛnza͡ɪm], \ɡlˈaɪkədʒən dˈɛbɹantʃɪŋ ˈɛnzaɪm], \ɡ_l_ˈaɪ_k_ə_dʒ_ə_n d_ˈɛ_b_ɹ_a_n_tʃ_ɪ_ŋ ˈɛ_n_z_aɪ_m]\
Definitions of GLYCOGEN DEBRANCHING ENZYME
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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 18.104.22.168) and amylo-1,6-glucosidase (EC 22.214.171.124) 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.
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- Complication diabetes from severe insulin deficiency coupled with an absolute or relative increase in concentration. metabolic acidosis is caused by breakdown of adipose stores and resulting increased levels free fatty acids. Glucagon accelerates the oxidation acids producing excess ketone bodies (ketosis).