A novel mutation in ACADVL causing very long-chain acyl-coenzyme-A dehydrogenase deficiency in a South Asian pediatric patient: a case report and review of the literature [PDF]
Journal of Medical Case Reports, 2021 Background Very long-chain acyl-coenzyme-A dehydrogenase deficiency is a rare, severe life-threatening metabolic disorder of mitochondrial fatty acid oxidation, caused by mutations in ACADVL gene.
Visvalingam Arunath +5 more
doaj +3 more sources
Progress of the acyl-Coenzyme A thioester hydrolase family in cancer [PDF]
Frontiers in OncologyIn recent years, the acyl-Coenzyme A thioester hydrolase family (ACOTs) has received wide attention as a key link in lipid metabolism. This family is a class of enzymes that catalyze the hydrolysis of fatty acyl-Coenzyme A, disrupting the thioester bond ...
Lu Bai +4 more
doaj +4 more sources
Acyl coenzyme A binding protein (ACBP): An aging- and disease-relevant "autophagy checkpoint". [PDF]
Aging Cell, 2023 Acyl coenzyme A binding protein (ACBP), also known as diazepam‐binding inhibitor (DBI), is a phylogenetically ancient protein present in some eubacteria and the entire eukaryotic radiation.
Montégut L +7 more
europepmc +2 more sources
Role of acyl-coenzyme A: cholesterol transferase 1 (ACAT1) in retinal neovascularization. [PDF]
J Neuroinflammation, 2023 Background We have investigated the efficacy of a new strategy to limit pathological retinal neovascularization (RNV) during ischemic retinopathy by targeting the cholesterol metabolizing enzyme acyl-coenzyme A: cholesterol transferase 1 (ACAT1 ...
Zaidi SAH +8 more
europepmc +2 more sources
Intratracheal Administration of Acyl Coenzyme A Acyltransferase-1 Inhibitor K-604 Reduces Pulmonary Inflammation Following Bleomycin-Induced Lung Injury. [PDF]
J Pharmacol Exp Ther, 2022 Acute lung injury (ALI) is characterized by epithelial damage, barrier dysfunction, and pulmonary edema. Macrophage activation and failure to resolve play a role in ALI; thus, macrophage phenotype modulation is a rational target for therapeutic ...
Stevenson ER +4 more
europepmc +2 more sources
FIT2 is an acyl-coenzyme A diphosphatase crucial for endoplasmic reticulum homeostasis. [PDF]
J Cell Biol, 2020 FIT2 is a protein important for ER lipid metabolism and lipid droplet formation, but its function has remained mysterious. Becuwe et al. show that FIT2 is an acyl coenzyme A diphosphatase, and this activity is crucial for ER homeostasis and cellular ...
Becuwe M +12 more
europepmc +2 more sources
Glycogen storage disease type Ia misdiagnosed as multiple acyl-coenzyme A dehydrogenase deficiency by mass spectrometry [PDF]
Frontiers in Pediatrics, 2022 ObjectiveTo report a case of glycogen storage disease (GSD) type Ia misdiagnosed as multiple acyl-coenzyme a dehydrogenase deficiency (MADD) by mass spectrometry.MethodsA 7 months old boy was admitted to our hospital for elevated transaminase levels ...
Juan Du +5 more
doaj +2 more sources
An update on the therapeutic implications of long-chain acyl-coenzyme A synthetases in nervous system diseases [PDF]
Frontiers in Neuroscience, 2022 Long-chain acyl-coenzyme A synthetases (ACSLs) are a family of CoA synthetases that activate fatty acid (FA) with chain lengths of 12–20 carbon atoms by forming the acyl-AMP derivative in an isozyme-specific manner.
Zhimin Wu +6 more
doaj +2 more sources
Journal of Lipid Research, 1987 Acyl coenzyme A:cholesterol acyl transferase and/or cholesterol esterase may regulate the esterification and absorption of exogenous cholesterol. To assess this, mucosal acyl coenzyme A:cholesterol acyl transferase activity was inhibited selectively with
L L Gallo, J A Wadsworth, G V Vahouny
doaj +2 more sources
Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency [PDF]
JA Clinical Reports, 2020 Background In a patient with very long-chain acyl-Coenzyme A dehydrogenase (VLCAD) deficiency, metabolism of fatty acids is impaired and a supply of alternative energy is limited when glucose level is insufficient on starvation.
Haruyuki Yuasa +5 more
doaj +2 more sources