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Glycogen is a highly branched glucose polymer consisting of chains of α(1->4) glycosyl bonds with α(1->6) glycosyl branch points. Glycogen is stored primarily in skeletal muscle and liver as a critical source of energy—in skeletal muscle as a fuel for muscle contraction, in liver as a source of glucose for extrahepatic metabolism. In both tissues, glycogen metabolism is achieved by the coordinated action of glycogen phosphorylase and debrancher. Glycogen phosphorylase splits α(1-4) glycosyl bonds to within 4 residues of a branch point. Glycogen debrancher (amylo-1,6-glucosidase,4-α-glucanotransferase [AGL]) has dual catalytic functions. First it transfers 3 of the final 4 residues at branch points to attach them in an α(1-4) linkage for metabolism by glycogen phosphorylase; then debrancher splits the α(1-6) bond to release the final glycosyl residue as free glucose.
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