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After studying this chapter, you should be able to:

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  • Describe the structure of glycogen and its importance as a carbohydrate reserve.
  • Describe the synthesis and breakdown of glycogen and how the processes are regulated in response to hormone action.
  • Describe the various types of glycogen storage diseases.

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Glycogen is the major storage carbohydrate in animals, corresponding to starch in plants; it is a branched polymer of α-D-glucose (Figure 14–13). It occurs mainly in liver and muscle, with modest amounts in the brain. Although the liver content of glycogen is greater than that of muscle, because the muscle mass of the body is considerably greater than that of the liver, about three-quarters of total body glycogen is in muscle (Table 19–1).

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Table Graphic Jump Location
Table 19–1 Storage of Carbohydrate in a 70 kg Human Being
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Muscle glycogen provides a readily available source of glucose 1-phosphate for glycolysis within the muscle itself. Liver glycogen functions to store and export glucose to maintain the blood glucose concentration in the fasting state. The liver concentration of glycogen is about 450 mM after a meal, falling to about 200 mM after an overnight fast; after 12–18 h of fasting, liver glycogen is almost totally depleted. Although muscle glycogen does not directly yield free glucose (because muscle lacks glucose 6-phosphatase), pyruvate formed by glycolysis in muscle can undergo transamination to alanine, which is exported from muscle and used for gluconeogenesis in the liver (Figure 20–4). Glycogen storage diseases are a group of inherited disorders characterized by deficient mobilization of glycogen or deposition of abnormal forms of glycogen, leading to liver damage and muscle weakness; some glycogen storage diseases result in early death.

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The highly branched structure of glycogen (Figure 14–13) provides a large number of sites for glycogenolysis, permitting rapid release of glucose 1-phosphate for muscle activity. Endurance athletes require a slower, more sustained release of glucose 1-phosphate. The formation of branch points in glycogen is slower than the addition of glucose units to a linear chain, and some endurance athletes practice carbohydrate loading—exercise to exhaustion (when muscle glycogen in largely depleted) followed by a high-carbohydrate meal, which results in rapid glycogen synthesis, with fewer branch points than normal.

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The Pathway of Glycogen Biosynthesis Involves a Special Nucleotide of Glucose

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As in glycolysis, glucose is phosphorylated to glucose 6-phosphate, catalyzed by hexokinase in muscle and glucokinase in liver (Figure 19–1). Glucose 6-phosphate is isomerized to glucose 1-phosphate by phosphoglucomutase. The enzyme itself is phosphorylated, and the phosphate group takes part in ...

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