Mitochondrial fatty acid oxidation (FAO) plays a pivotal role in maintaining body energy
homoeostasis mainly during catabolic states. Oxidation of fatty acids requires approximately …

Oxidation of fatty acids in mitochondria is a key physiological process in higher eukaryotes
including humans. The importance of the mitochondrial beta-oxidation system in humans is …

Published data on treatment of fatty acid oxidation defects are scarce. Treatment
recommendations have been developed on the basis of observations in 75 patients with …

In Taiwan, during the period March 2000 to June 2009, 1,495,132 neonates were screened
for phenylketonuria (PKU) and homocystinuria (HCU), and 1,321,123 neonates were …

Fatty acid oxidation disorders (FAODs) are inborn errors of metabolism (IEMs) caused by
defects in the fatty acid (FA) mitochondrial β-oxidation. The most common FAODs are …

Mitochondrial fatty acid oxidation disorders (FAODs) are caused by defects in β-oxidation
enzymes, including very long-chain acyl-CoA dehydrogenase (VLCAD), trifunctional protein …

The different long-chain fatty acid oxidation defects present with similar heterogeneous
clinical phenotypes of different severity. Organs mainly affected comprise the heart, liver …

At present, long‐chain fatty acid oxidation (FAO) defects are diagnosed in a number of
countries by newborn screening using tandem mass spectrometry. In the majority of cases# …

Abstract Treatment recommendations in mitochondrial fatty acid oxidation (FAO) defects are
diverse. With implementation of newborn screening and identification of asymptomatic …

Introduction: Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a disorder of
oxidation of long chain fat, and can present as cardiomyopathy or fasting intolerance in the …