9 documents found, page 1 of 1

Guanidinoacetate methyltransferase deficiency identified in adults and a child ...

Our study describes the adult clinical and biochemical spectrum of guanidinoacetate methyltransferase (GAMT) deficiency, a recently discovered inborn error of metabolism. The majority of the previous reports dealt with pediatric patients, in contrast to t

Characterization of fatty acylcarnitines in rat hepatocytes in vitro

Valproyl-dephosphocoa

The mitochondrial metabolism of valproic acid (VPA) was investigated in vitro to elucidate its beta-oxidation pathway since the characterization of VPA intermediates in the acyl-CoA thioester form, and not just in their free acid form, has not been fully

Complete beta-oxidation of valproate

The beta-oxidation of valproic acid (VPA; 2-n-propylpentanoic acid) was investigated in vitro in intact rat liver mitochondria incubated with H-3-labelled VPA. The metabolism of [4,5-H-3(2)]VPA and [2-H-3]VPA was studied by analysing the different acyl-Co

Valproate induces in vitro accumulation of long-chain fatty acylcarnitines

To elucidate the interference mechanisms of valproate (VPA) with mitochondrial fatty acid beta -oxidation (FAO), the profile of acylcarnitine formation was studied in vitro. Human fibroblasts were incubated with 0.2 mmol/L [U-C-13]palmitate, 0.4 mmol/L L-

Differential effect of valproate and its Delta(2)- and Delta(4)-unsaturated met...

Overall fatty acid oxidation rates were investigated in rat hepatocytes using [9,10-H-3]palmitic, [9,10-H-3]-oleic, [9.10-H-3]-myristic and [2,3-H-3]-phenylpropionic acids. The effect of both valproate (VPA) (0-10 mM) and two of its unsaturated metabolite

Characterization of plasma acylcarnitines in patients under valproate monothera...

Objectives: The effect of administration of the antiepileptic drug valproate (VPA), on the composition of the plasma acylcarnitine profile (including free carnitine) was investigated. Design and methods: Plasma samples were obtained from 18 individuals (1

Synthesis and intramitochondrial levels of valproyl-coenzyme A metabolites

A number of valproate adverse reactions are due to its interference with several metabolic pathways, including that of fatty acid oxidation. In order to resolve which mitochondrial enzymes of fatty acid oxidation are inhibited by which VPA intermediates w

Quantitative analysis of urinary acylglycines for the diagnosis of beta-oxidati...

The analysis of acylglycines is an important biochemical tool for the diagnosis of inherited disorders of mitochondrial fatty acid P-oxidation. A stable isotope dilution gas chromatography negative chemical ionisation mass spectrometry method for the quantitative analysis of short and medium-chain acylglycines as their bis(trifluoromethyl)benzyl (BTFMB) ester derivatives is described. The diagnostic usefulness ...