BACKGROUND Carnitine deficiency may contribute to cardiovascular disease (CVD) in patients with hemodialysis (HD). Dyslipidemia plays a role in CVD and its prevalence is also high in HD patients. We examined here the effects of switching from oral administration (PO) to intravenous (IV) injection of l-carnitine on lipid metabolism in patients with HD. METHODS Nine HD patients who had received...

Background: Type 1 diabetes mellitus is reportedly characterized by deficiencies of plasma total and free carnitine. Several inherited defects in fatty acid oxidation are associated with carnitine deficiency and with recurrent hypoglycemia. Hypoglycemia continues to be the major limiting factor in the management of type 1 diabetes. Herein, we sought to identify a subset of patients with abnorma...

BACKGROUND AND AIMS Atherosclerotic cardiovascular disease is the most common cause of mortality in patients with end-stage kidney disease. Chronic kidney disease patients often exhibit a deficiency in L-carnitine due to loss during hemodialysis (HD). We studied the effects of L-carnitine supplementation on brachial-ankle pulse wave velocity (baPWV), a marker of atherosclerosis, in HD patients....

AIM This study was performed to test the hypothesis that urinary levels of L-carnitine and its derivatives are enhanced in children and adolescents with hypertension and also check if analyzed parameters may serve as early markers of subclinical renal damage. METHODS The study included 112 children and adolescents (67 males and 45 females) aged median 10-18 years. Participants were divided in...

AIMS To investigate the effect of carnitine supplementation on alcoholic malnourished rats' hepatic nitrogen content. METHODS Malnourished rats, on 50% protein-calorie restriction with free access to water (malnutrition group) and malnourished rats under the same conditions with free access to a 20% alcohol/water solution (alcohol group) were studied. After the undernourishment period (4 week...

This study was conducted to investigate developmental changes in the kinetics of carnitine palmitoyltransferase (CPT) within hepatic and skeletal muscle tissues of the canine species. Carnitine concentrations, CPT activity and the apparent K(m) for carnitine were measured in tissue homogenates from dogs in six age categories: newborn; 24-h-old; 3-, 6- and 9-wk-old; and adult. Hepatic CPT activi...

The kinetic behavior of the carnitine:acylcarnitine translocase was studied in isolated rat heart mitochondria. The kinetic parameters, Km(apparent) and Vmax, for carnitine were determined by measuring the rates of influx of [14C]carnitine using two different methods to quench the exchange reaction. The range of the Km(app) was 0.38-1.50 mM and the Vmax was 0.20-0.34 nmol/mg . min by both metho...

BACKGROUND The prevalence of primary carnitine deficiency (PCD) in the Faroe Islands is the highest reported in the world (1:300). Serious symptoms related to PCD, e.g. sudden death, have previously only been associated to the c.95A > G/c.95A > G genotype in the Faroe Islands. We report and characterize novel mutations associated with PCD in the Faroese population and report and compare free ca...

The purpose of this study was to investigate the cellular distribution of carnitine and its acyl derivatives in the normal and ischemic myocardium, and the effects of exogenous 1-carnitine on this distribution and mitochondrial function in the ischemic dog heart. Under non-ischemic conditions, about 93% of the total cellular carnitine was located in the cytosolic compartment and 6.5% in the mit...