Merck Manual

Please confirm that you are a health care professional

honeypot link

Overview of Fatty Acid and Glycerol Metabolism Disorders

By

Matt Demczko

, MD, Sidney Kimmel Medical College of Thomas Jefferson University

Last full review/revision Apr 2020| Content last modified Apr 2020
Click here for 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

Table
icon
Table
icon
Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Professionals also read
Test your knowledge
Congenital Limb Abnormalities
Congenital limb amputations and deficiencies are missing or incomplete limbs at birth. There are many possible causes of congenital limb amputations, and they often occur as a component of various congenital syndromes. Which of the following causes of these amputations is most common?
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID

Also of Interest

Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
TOP