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9: Disorders of Monosaccharide Metabolism and Other Metabolic Conditions

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    Learning objectives

    Monosaccharide metabolism

    1. Describe fructose and galactose metabolism, including the location of the pathway, substrate, and regulatory enzymes.
    2. Apply knowledge of metabolic regulation to determine how deficiencies in monosaccharide metabolism lead to hypoglycemia.

    Alcohol metabolism

    1. Determine clinical consequences of increased NADH on the metabolic pathways of \(\beta\)-oxidation and gluconeogenesis.
    2. Describe the two-step process of alcohol metabolism by ADH and ALDH.

    Metabolism to this point has focused on the oxidation of carbohydrates (primarily glucose), lipids, and amino acids. We have addressed these processes under normal conditions without much consideration for potential interactions of other metabolites or pathologies. This section will address how some additional situations can impact the metabolism of these macromolecules. First, metabolism of both galactose and fructose is important and will be addressed. Heritable deficiencies in either of these pathways can potentially cause severe symptoms, and learning how to identify key symptoms is essential. Metabolism of alcohol is also a process to be aware of, as excess flux through this pathway has the potential to interfere with normal glucose and lipid metabolism.

    Thumbnail: Grey, Kindred, Chapter 9. 2021. CC BY 4.0

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