Article
Pathophysiology of fatty acid oxidation disorders.
Journal of inherited metabolic disease - 1 Oct 2010
Bennett M J
Abstract excerpt
Mitochondrial fatty acid oxidation represents an important pathway for energy generation during periods of increased energy demand such as fasting, febrile illness and muscular exertion. In liver, the primary end products of the pathway are ketone bodies, which are released into the circulation and provide energy to tissues that are not able to oxidize fatty acids such as brain. Other tissues, such as cardiac and...
Topics
- Animals
- Energy Metabolism
- Fatty Acids
- Genotype
- Humans
- Lipid Metabolism, Inborn Errors
- Mitochondria
- Mitochondrial Diseases
- Oxidation-Reduction
- Phenotype
