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
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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
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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
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
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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
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A rapid assay of acyl-coenzyme A:lysolecithin acyltransferase activity
Journal of Lipid Research, 1980 A simple and rapid procedure for the assay of acyl-coenzyme A:1-acyl-sn-glycero-3-phosphocholine acyltransferase (lysolecithin acyltransferase, LLAT [EC 2.3.1.23]) activity in crude enzyme preparations is described.
K Hayase +3 more
doaj +4 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
Acyl-coenzyme A:cholesterol acyltransferases [PDF]
American Journal of Physiology-Endocrinology and Metabolism, 2009 The enzymes acyl-coenzyme A (CoA):cholesterol acyltransferases (ACATs) are membrane-bound proteins that utilize long-chain fatty acyl-CoA and cholesterol as substrates to form cholesteryl esters. In mammals, two isoenzymes, ACAT1 and ACAT2, encoded by two different genes, exist.
Bo-Liang Li +3 more
openaire +3 more sources
A FRET sensor for the real-time detection of long chain acyl-CoAs and synthetic ABHD5 ligands
Cell Reports: Methods, 2023 Summary: Intracellular long-chain acyl-coenzyme As (LC-acyl-CoAs) are thought to be under tight spatial and temporal controls, yet the ability to image LC-acyl-CoAs in live cells is lacking.
Emilio P. Mottillo +6 more
doaj +1 more source
Acyl‐coenzyme A organizes laterally in membranes and is recognized specifically by acyl‐coenzyme A binding protein [PDF]
FEBS Letters, 2003 Long chain acyl‐coenzyme A (acyl‐CoA) is a biochemically important amphiphilic molecule that is known to partition strongly into membranes by insertion of the acyl chain. At present, microscopically resolved evidence is lacking on how acyl‐CoA influences and organizes laterally in membranes. By atomic force microscopy (AFM) imaging of membranes exposed
Cohen Simonsen, A. +4 more
openaire +5 more sources