Isobutyryl-L-carnitine is a product of the acyl-CoA dehydrogenases (ACADs), which are a group of mitochondrial enzymes involved in the metabolism of fatty acids or branched-chain amino acids.
A large quantity of propionylcarnitine in the urine of patients with propionic acidemia and methylmalonic aciduria was demonstrated. The amount excreted depended on the administered L-carnitine dose from 25 to 75 mg/kg/day. A high level of propionylcarnitine was also detected
European journal of pediatrics, 153(8), 598-603 (1994-08-01)
We describe on a 3-year-old child referred for evaluation and therapy of a cerebral vascular accident with residual hemiplegia and partial epilepsy. Metabolic investigations initially showed normal urinary organic acids as well as normal blood and urinary amino acids. Blood
Clinica chimica acta; international journal of clinical chemistry, 216(1-2), 135-143 (1993-07-16)
An immobilized enzyme reactor, made up acylcarnitine hydrolase, carnitine dehydrogenase and diaphorase in sequence, was developed for the sensitive and selective determination of urinary free and individual acylcarnitines by a reversed-phase high-performance liquid chromatography. A 100-microliter urine sample was directly
Tandem mass spectrometry was adopted for newborn screening by North Carolina in April 1999. Since then, three infants with short-chain acyl-CoA dehydrogenase (SCAD) and one with isobutyryl-CoA dehydrogenase deficiency were detected on the basis of elevated butyrylcarnitine/isobutyrylcarnitine (C4-carnitine) concentrations in
When washed human sperm were incubated in a modified Krebs-Ringer buffer solution in the absence of exogenous metabolizable substrates at 30 degrees C they maintained progressive motility for at least six hours. Under these conditions the spermatozoa apparently utilize endogenous
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