TY - CHAP M1 - Book, Section TI - Glycogen Storage Diseases and Other Inherited Disorders of Carbohydrate Metabolism A1 - Kishnani, Priya S. A1 - Chen, Yuan-Tsong A2 - Jameson, J. Larry A2 - Fauci, Anthony S. A2 - Kasper, Dennis L. A2 - Hauser, Stephen L. A2 - Longo, Dan L. A2 - Loscalzo, Joseph PY - 2018 T2 - Harrison's Principles of Internal Medicine, 20e AB - 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. 412-1. Glucose is the principal 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 (e.g., lactose, maltose, and sucrose). Lactose 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. Lactose is derived from galactose + glucose, which is found in milk products, and is an important component of 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspharmacy.mhmedical.com/content.aspx?aid=1160017961 ER -