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Harrison's Principles of Internal Medicine, 18e | Part 16. Endocrinology and Metabolism > Section 3. Disorders of Intermediary Metabolism > | Chapter 362. Glycogen Storage Diseases and Other Inherited Disorders of Carbohydrate Metabolism Sections: Glycogen Storage Diseases and Other Inherited Disorders of Carbohydrate Metabolism: Introduction, Selected Liver Glycogenoses, Selected Muscle Glycogenoses, Selected Disorders of Galactose Metabolism, Selected Disorders of Fructose Metabolism, Global Considerations, Further Readings. Topics Discussed: carbohydrate metabolism disorder; carbohydrate metabolism, inborn errors; glycogen storage disease. Excerpt:"Carbohydrate metabolism plays a vital role in cellular function by providing the energy required for most metabolic processes. The relevant biochemical pathways involved in the metabolism of these carbohydrates are shown in Fig. 362-1. Glucose is the principle substrate of energy metabolism in humans. Metabolism of glucose generates ATP through glycolysis and mitochondrial oxidative phosphorylation. The body obtains glucose through the ingestion of polysaccharides, primarily starch, and disaccharides including lactose, maltose, and sucrose. Galactose and fructose are two other monosaccharides that serve as sources of fuel for cellular metabolism; however, their role as fuel sources is much less significant than that of glucose. Galactose is derived from lactose (galactose + glucose), which is found in milk products, and is an important component for certain glycolipids, glycoproteins, and glycosaminoglycans. Fructose is found in fruits, vegetables, and honey. Sucrose (fructose + glucose) is another dietary source of fructose and is a commonly used sweetener...."
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