in the 1980’s, medium-chain acyl-coa dehydrogenase deficiency (mcadd) was first described in the literature as three children who presented with coma, hypoglycemia, hyperammonemia, and fatty liver while fasting. these symptoms while similar to reye’s syndrome, were found to be due to an inability to metabolize medium chain fatty acids during fasting periods. fatty acids are utilized by the body...

It is shown that acyl-CoA binding protein (ACBP), in contrast with fatty acid binding protein (FABP), stimulates the synthesis of long-chain acyl-CoA esters by mitochondria. ACBP effectively opposes the product feedback inhibition of the long-chain acyl-CoA synthetase by sequestration of the synthesized acyl-CoA esters. Feedback inhibition of microsomal long-chain acyl-CoA synthesis could not b...

To determine whether accumulation of long-chain acyl carnitine contributes to electrophysiological abnormalities induced by hypoxia, we characterized effects of normoxic and hypoxic perfusion on the subcellular distribution of endogenous long-chain acyl carnitine and transmembrane potentials of cultured rat neonatal myocytes. Hypoxia increased long-chain acyl carnitine more than 5-fold. Sodium ...

To determine whether accumulation of long-chain acyl camitine contributes to electrophysiological abnormalities induced by hypoxia, we characterized effects of normoxic and hypoxic perfusion on the subcellular distribution of endogenous long-chain acyl camitine and transmembrane potentials of cultured rat neonatal myocytes. Hypoxia increased long-chain acyl camitine more than 5-fold. Sodium 2-[...

metabolic myopathies are genetically inherited disorders of muscle energy production that result in skeletal muscle dysfunction. they are a large group of diseases with diverse inborn errors of metabolism, in particular muscle energy production, and including disorders of glycogen (lysosomal and non-lysosomal glycogenoses), lipid (disorders of fatty acid b-oxidation, primary carnitine deficienc...

Long-chain acyl-acyl carrier proteins (acyl-ACP) are established biochemical regulators of bacterial type II fatty acid synthases due to their ability to feedback-inhibit the early steps in the biosynthetic pathway. In Streptococcus pneumoniae, the expression of the fatty acid synthase (fab) genes is controlled by a helix-turn-helix transcriptional repressor called FabT. A screen of pathway int...

The deuterium nuclear magnetic resonance (2H NMR) spectrum of perdeuterated tetradecanol in a mixture of 1-palmitoyl-2-oleoyl-phosphatidylethanolamine (POPE) and water was used to compare the variation of the acyl chain orientational order parameter, S(n), with carbon position, n, in the liquid crystalline lamellar (La) and hexagonal (HI,) phases. The characteristic independence of S(n) with n ...

background: n-acyl homoserine lactone (ahl) is found to be the main component of quorum sensing (qs) in gram-negative bacteria and plays an important role in biofilm formation. little information is available regarding the role of ahl in biofilm formation in escherichia coli (e. coli). the purpose of this investigation was to biochemically detect and characterize ahl activity in biofilm-forming...

Three straight chain acyl-CoA dehydrogenases were purified to apparent homogeneity from bovine liver using 40-70% (NH4)2SO4 precipitation, gel filtration, DEAE-cellulose column chromatography, and preparative electrophoresis. Separation of the acyl-CoA dehydrogenases by these procedures has been efficiently monitored by two newly developed analytical methods: (i) native staining of acyl-CoA deh...

Acyl arbutin was synthesized through the condensation of arbutin with a saturated fatty acid (C6-18) by the immobilized lipase in a batch reaction. The conversion at 10 and 20 g/l-solvent of immobilized lipase reached 45% over 2 d, but the initial reaction rate per amount of immobilized lipase decreased at 20 g/l-solvent. The radical scavenging activity of acyl arbutin in an ethanol solution wa...