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 …

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 …

The mitochondrial sirtuin SIRT3 is a protein deacylase that influences almost every major
aspect of mitochondrial biology, including nutrient oxidation, ATP generation, reactive …

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

Background—Carnitine palmitoyltransferase-1 (CPT1) is a rate-limiting step of mitochondrial
β-oxidation by controlling the mitochondrial uptake of long-chain acyl-CoAs. The muscle …

The main hallmark of myocardial substrate metabolism in cardiac hypertrophy or heart
failure is a shift from fatty acid oxidation to greater reliance on glycolysis. However, the close …

Long-chain acyl coenzyme A (acyl-CoA) synthetase isoform 1 (ACSL1) catalyzes the
synthesis of acyl-CoA from long-chain fatty acids and contributes the majority of cardiac long …

The heart's extraordinary metabolic flexibility allows it to adapt to normal changes in
physiology in order to preserve its function. Alterations in the metabolic profile of the heart …

In eukaryotes, mitochondria carry out numerous functions that are central to cellular and
organismal health. How mitochondrial activities are regulated in response to differing …

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