Mitochondrial fatty acid β-oxidation (FAO) is the major pathway for the degradation of fatty
acids and is essential for maintaining energy homeostasis in the human body. Fatty acids …

In recent years increasing evidence has emerged suggesting that oxidative stress is
involved in the pathophysiology of a number of inherited metabolic disorders. However the …

Emerging evidence suggests that protein acetylation is a broad-ranging regulatory
mechanism. Here we utilize acetyl-peptide arrays and metabolomic analyses to identify …

Objective Metabolic reprogramming of tumour cells that allows for adaptation to their local
environment is a hallmark of cancer. Interestingly, obesity-driven and non-alcoholic …

l-Carnitine is an amino acid derivative widely known for its involvement in the transport of
long-chain fatty acids into the mitochondrial matrix, where fatty acid oxidation occurs …

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 …

Inherited defects in mitochondrial fatty-acid beta-oxidation comprise a group of at least 12
diseases characterized by distinct enzyme or transporter deficiencies. Most of these …

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# …

Acylcarnitines are intermediates of fatty acid and amino acid oxidation found in tissues and
body fluids. They are important diagnostic markers for inherited diseases of peroxisomal and …

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