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Chapter 34 - Inborn Errors of Fatty Acid Oxidation

from Section IV - Metabolic Liver Disease

Published online by Cambridge University Press:  19 January 2021

By
Jerry Vockley
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
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Summary

Mitochondrial fatty acid β-oxidation (FAO) is an essential component of energy production and homeostasis in humans. During periods of limited glucose supply, FAO in the liver provides energy for hepatic function and the acetyl-CoA substrate needed for hepatocytes to synthesize and release ketone bodies into circulation. Ketone bodies provide an alternative energy substrate for peripheral tissues when glucose supply is limited. Other tissues such as skeletal and cardiac muscle rely on FAO for energy production. The oxidation of fatty acids can provide up to 80% of the energy requirements for cardiac and skeletal muscle while sparing glucose for use by the brain and CNS during moderate exercise, fasting, or illness. Disorders in the ability to use fatty acids for energy production manifest during periods of increased energy demands or reduced caloric intake.

Type
Chapter
Information
Liver Disease in Children , pp. 611 - 627
Publisher: Cambridge University Press
Print publication year: 2021

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