Fatty-acid metabolism disorder

A broad classification for genetic disorders that result from an inability of the body to produce or utilize an enzyme or transport protein that is required to oxidize fatty acids.

This leaves the body unable to produce energy within the liver and muscles from fatty acid sources.

[1] Most individuals with a fatty-acid metabolism disorder are able to live a normal active life with simple adjustments to diet and medications.

When in need of glucose the body of a person with a fatty-acid metabolism disorder will still send fats to the liver.

Fatty-acid metabolism disorders result when both parents of the diagnosed subject are carriers of a defective gene.

[citation needed] Normally, in cases of hypoglycaemia, triglycerides and fatty acids are metabolised to provide glucose/energy.

Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of LCHAD deficiency
Transport