glycogen debranching enzyme

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A monomeric protein (1515 aa) with two catalytic activities, amylo-1,6-glucosidase (EC and 4-α-glucanotransferase (EC, with different catalytic sites. Both activities are necessary to break down branched regions of glycogen. Deficiencies in the enzyme lead to glycogen storage disease III.

Subjects: Medicine and Health.

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