Carnitine deficiency occurring in families has been rarely reported and the genetic transmission has not yet been clearly elucidated. Five members of one family showing marked heterogeneity of carnitine deficiency states are presented. In three patients, there was no correlation between measurable carnitine levels in serum and muscle and the clinical findings. The parents, who are remote relati...

PURPOSE To investigate the pharmacokinetics of L-carnitine (LC) and its analogues, acetyl-L-carnitine (ALC) and propionyl-L-carnitine (PLC) in healthy volunteers after single L-carnitine administration. METHODS Liquid L-carnitine (2.0 g) was administered orally as a single dose in 12 healthy subjects. Plasma and urine concentrations of L-carnitine, ALC and PLC were detected by HPLC. RESULTS...

background: metabolic derangements in type 2 diabetes mellitus (t2dm) are likely to affect skeletal muscle contractile functions adversely. levo-carnitine improves muscle contractile functions in healthy humans and rats and corrects metabolic derangements in t2dm. therefore, it is likely to improve muscle contractile functions in t2dm as well. this study was designed to determine the effect of ...

Carnitine is needed for a variety of important physiological functions in energy metabolism. Assessment of the carnitine status of an individual is compromised by limited data on the number of metabolic compartments of carnitine and their interrelationship, if any. Possible compartmentalization of carnitine in the blood of healthy adults was investigated because blood is one of the more readily...

In this study, we sought to identify the transporters that mediate the uptake of L-carnitine and acetyl-L-carnitine in cultured rat cortical astrocytes. L-[(3)H]carnitine and acetyl-L-[(3)H]carnitine uptake were both saturable, and mediated by a single Na(+)-dependent transport system. Uptake of both was inhibited by L-carnitine, D-carnitine, acetyl-L-carnitine and various organic cations. Acyl...

Growth performance, food efficiency and body composition of pikeperch (Sander lucioperca) were investigated with 1 or 2 g/kg L-carnitine added to the diet. Control diet did not contain L-carnitine. Two hundred pikeperch fingerlings (1.63 g, mean weight) were stocked in each 1 m3 concrete tank and fed equally 6 meals per day for 6 weeks. Higher increment in body weight (5.92 ± 0.37 g), the ...

The metabolic function of carnitine in the fatty acid oxidase-deficient flight muscle of the blowfly, Phormia regina, was investigated. Mitochondria from flies did not oxidize pahnitate or hexanoate in the presence of adenosine triphosphate, coenzyme A, carnitine, and serum albumin. Pahnitoyl carnitine was oxidized at an extremely slow rate, QOz of 20. The rate of synthesis of the carnitine est...

Carnitine has important roles in skeletal muscle bioenergetics. Skeletal muscle carnitine deficiency is associated with profound impairment of muscle function. It has thus been natural to ask if carnitine supplementation can improve skeletal muscle function and athletic performance in healthy individuals. Oral carnitine doses of several grams cause no significant clinical toxicity, further enco...

have been reported by several authors [2,3,6 ]. The Introduction high total serum concentration of carnitine results predominantly from an increase in -carnitine esters, Recombinant human erythropoietin (rhEpo) is widely i.e. acylcarnitine. As a result of the reduction in free used for correction of anaemia in patients with endcarnitine, the ratios of free/total carnitine and free stage renal ...