glycogen debranching enzyme systemmedical dictionary

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 and amylo-1,6-glucosidase (ec 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 catalyses 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.

(12 Dec 1998)