L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.
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L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.
Treatment with L-carnitine greatly enhanced the formation and excretion of short-chain acylcarnitines in three patients with propionic acidemia and in three normal controls. The use of fast atom bombardment mass spectrometry and linked scanning at constant magnetic (B) to electric (E) field ratio identified the acylcarnitine as propionylcarnitine in patients with propionic acidemia. The normal ...
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492 CLINICAL CHEMISTRY, Vol.31, No. 3, 1985 1. Fishlock RC, Bieber LL, Snoswell AM. Sources of error in determinations of carnitine and acylcarnitine in plasma. Clin Chem 30, 316-318 (1984). 2. Brass EP, Hoppel CL. Carnitine metabolism in the fasting rat. J Biol Chem 253, 2688-2693 (1978). 3. Genuth SM, Hoppel CL. Plasma and urine carnitine in diabetic ketosis. Diabetes 28, 1083-1087 (1978). 4....
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Propionyl-L-carnitine (PLC) is an ester of L-carnitine (LC) under evaluation for the treatment of cardiovascular disorders. The renal disposition of PLC was studied in the isolated perfused rat kidney with deuterium-labeled derivative (PLC-CD3). Kidneys of male Sprague-Dawley rats were perfused at initial PLC-CD3 concentrations of 10 (n = 4) and 200 microM (n = 5). High-performance liquid chrom...
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In the search for the mechanism by which hyperammonemia complicates propionic and methylmalonic acidemia the effects of a series of acyl-coenzyme A (CoA) derivatives were studied on the activity of N-acetylglutamate synthetase in rat liver mitochondria using acetyl-CoA as substrate. Propionyl-CoA was found to be a competitive inhibitor. The inhibition constant of 0.71 mM is in the range of conc...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1984
ISSN: 0021-9738
DOI: 10.1172/jci111387