A general introduction to the biochemistry of mitochondrial fatty acid β-oxidation

By Sander M. Houten, R. Wanders. Journal of Inherited Metabolic Disease, 2010

938 citations recorded by the source corpus.

Over the years, the mitochondrial fatty acid β-oxidation (FAO) pathway has been characterised at the biochemical level as well as the molecular biological level. FAO plays a pivotal role in energy homoeostasis, but it competes with glucose as the primary oxidative substrate. The mechanisms behind this so-called glucose–fatty acid cycle operate at the hormonal, transcriptional and biochemical levels. Inherited defects for most of the FAO enzymes have been identified and characterised and are currently included in neonatal screening programmes. Symptoms range from hypoketotic hypoglycaemia to skeletal and cardiac myopathies. The pathophysiology of these diseases is still not completely understood, hampering optimal treatment. Studies of patients and mouse models will contribute to our understanding of the pathogenesis and will ultimately lead to better treatment.

Subfields: Clinical Biochemistry, Molecular Biology, Physiology

View Paper: https://www.semanticscholar.org/paper/2ace922fb3b29796bf905b92776cc5541bef5aaf

Citations in the corpus

• CARNITINE TRANSPORT AND FATTY ACID OXIDATION (2016, neutral): “FATPs have acyl-CoA synthase (ACS) activity [20], converting fatty acids to acyl-CoA thioesters in order to activate them for metabolic processes [2, 20, 22].”