13 documents found, page 1 of 2

Intracellular S-adenosylhomocysteine increased levels are associated with DNA h...

Hyperhomocysteinemia is a risk factor for atherosclerosis and vascular disease; however, the mechanism underlying this association remains poorly understood. Increased levels of intracellular S-adenosylhomocysteine (AdoHcy), secondary to homocysteine-medi

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

5,10-methylenetetrahydrofolate reductase (MTHFR) 677C - T and 1298A - C mutatio...

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

Increased, homocysteine and S-adenosylhomocysteine concentrations and DNA hypom...

Background: The pathogenic mechanism of homocysteine's effect on cardiovascular risk is poorly understood. Recent studies show that DNA hypomethylation induced by increases in S-adenosylhomocysteine (AdoHcy), an intermediate of Hcy metabolism and a potent

5,10-Methylenetetrahydrofolate reductase 677C - T and 1298A - C mutations are g...

Background: Methylenetetrahydrofolate reductase (MTHFR) is one of the main regulatory enzymes of homocysteine metabolism. Elevated plasma total homocysteine (tHcy) is a major risk for cardiovascular disease. A common 677C--T mutation in the MTHFR gene re

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

Quantification of plasma S-adenosylmethionine and S-adenosylhomocysteine as the...

A simplified reversed phase HPLC system for the detection of fluorescent 1,N-6-etheno derivatives of SAM (S-adenosylmethionine) and S-adenosylhomocysteine (SAH) is described. The most important changes from the previously reported method are a shorter der

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