Stearoylcarnitine is found in significantly greater amounts in patients with carnitine palmitoyltransferase II deficiency. Carnitine palmitoyltransferase, in conjunction with acyl-CoA synthetase and carnitine/acylcarnitine translocase, provides the mechanism whereby long-chain fatty acids are transferred from the cytosol to the mitochondrial matrix to undergo beta-oxidation.
Lipid infusion or ingestion of a high-fat diet results in insulin resistance, but the mechanism underlying this phenomenon remains unclear. Here we show that, in rats fed a high-fat diet, whole-animal, muscle and liver insulin resistance is ameliorated following hepatic
Global profiling of the muscle metabolome: method optimization, validation and application to determine exercise-induced metabolic effects.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 792(1), 63-72 (2003-06-28)
In a selective screening for fatty acid oxidation disorders by tandem mass spectrometry, we tested the diagnostic ratios and acylcarnitine concentrations in sera or blood spots, which were reported to be specific to very long-chain acyl CoA dehydrogenase deficiency, carnitine
Analytical and bioanalytical chemistry, 375(2), 200-210 (2003-02-01)
l-Carnitine and its esters are products of intermediary metabolism of organisms. The distribution pattern or the favored excretion of individual acylcarnitines tells something about metabolic diseases. The determination of the urinary acylcarnitine pattern by flow injection analysis (FIA)-electrospray ionization (ESI)-mass
Acylcarnitine profiling from blood or plasma samples by electrospray tandem mass spectrometry (ESI-MS/MS) has been recognized recently as a useful tool in the biochemical diagnosis of propionic acidemia, methylmalonic acidemia together with short-chain and medium-chain acyl-CoA dehydrogenase deficiencies. In the
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