Merck Manual

Please confirm that you are a health care professional

honeypot link

Overview of Fatty Acid and Glycerol Metabolism Disorders


Matt Demczko

, MD, Mitochondrial Medicine, Children's Hospital of Philadelphia

Last review/revision Oct 2021 | Modified Sep 2022
View Patient Education
Topic Resources

Fatty acids are the preferred energy source for the heart and an important energy source for skeletal muscle during prolonged exertion. Also, during fasting, the bulk of the body’s energy needs must be supplied by fat metabolism. Using fat as an energy source requires catabolizing adipose tissue into free fatty acid and glycerol. The free fatty acid is metabolized in the liver and peripheral tissue via beta-oxidation into acetyl CoA; the glycerol is used by the liver for triglyceride synthesis or for gluconeogenesis. Carnitine is required for long-chain fatty acid oxidation. Carnitine deficiencies Carnitine Deficiency Carnitine deficiency results from inadequate intake of or inability to metabolize the amino acid carnitine. It can cause a heterogeneous group of disorders. Muscle metabolism is impaired, causing... read more can be primary or secondary. Secondary carnitine deficiency is a secondary biochemical feature of many organic acidemias and fatty acid oxidation defects.

There are a number of other disorders of fatty acid and glycerol metabolism, including those involving


More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

View Patient Education
NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
quiz link

Test your knowledge

Take a Quiz!