Acylcarnitines are fatty acid metabolites that play important roles in many cellular energy
metabolism pathways. They have historically been used as important diagnostic markers for …

Mitochondrial fatty acid oxidation is an essential pathway for energy production, especially
during prolonged fasting and sub-maximal exercise. Long-chain fatty acids are the most …

Fatty acid oxidation disorders (FAODs) are inborn errors of metabolism due to disruption of
either mitochondrial β-oxidation or the fatty acid transport using the carnitine transport …

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

Peroxisomes and mitochondria are ubiquitous, highly dynamic organelles with an oxidative
type of metabolism in eukaryotic cells. Over the years, substantial evidence has been …

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 …

BACKGROUND: Although extended newborn screening (NBS) programs have been
introduced more than 20 years ago, their impact on the long-term clinical outcome of …

Malnutrition and sarcopenia are common in patients with chronic liver disease and are
associated with increased risk of decompensation, infections, wait-list mortality and poorer …

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 …