L-carnitine is a quaternary amine that is essential for the normal oxidation of long-chain fatty acids by mitochondria. It is known that L-carnitine and its derivatives prevent the formation of reactive oxygen species, scavenge free radicals and protect cells from peroxidative stress. Oxygen-derived free radicals and lipid peroxidation products play a critical role in the pathogenesis of ethano...

A random retrospective chart review was conducted to document serum carnitine levels on 100 children with autism. Concurrently drawn serum pyruvate, lactate, ammonia, and alanine levels were also available in many of these children. Values of free and total carnitine (p < 0.001), and pyruvate (p = 0.006) were significantly reduced while ammonia and alanine levels were considerably elevated (p <...

The radioisotopic assay for carnitine first described by Cederblad and Lindstedt (Clin. Chim. Acta. 37:235-543, 1972) and modified by Bohmer et al (Clin. Chim. Acta. 57:55-61, 1974) has been improved and simplified. As a result, the assay yields a linear response over a wide range of carnitine concentrations without the need for excessive amounts of labeled acetyl-CoA. In addition, it will meas...

It is well documented that high therapeutic doses of oxazaphosphorines, cyclophosphamide (CP) and ifosfamide (IFO), are associated with cardiomyopathy. This study investigated whether oxazaphosphorines alter the expression of organic cation/carnitine transporter (OCTN2) and antioxidant genes and if so, whether these alterations contribute to CP and IFO-induced cardiotoxicity. Adult male Wistar ...

Two groups of guinea pigs received 1% ascorbic acid in their drinking water to achieve tissue saturation and were afterwards maintained on an ascorbic acid-free regime till death. One group received fresh, aqueous DL-carnitine (lOmg/ml/kg body weight) daily, the other an equivalent volume of water. The carnitine-supplemented group survived for a significantly longer period than the non-suppleme...

Excess fat accumulation has been observed widely in farmed fish; therefore, efficient lipid-lowering factors have obtained high attention in the current fish nutrition studies. Dietary L-carnitine can increase fatty acid β-oxidation in mammals, but has produced contradictory results in different fish species. To date, the mechanisms of metabolic regulation of L-carnitine in fish have not been f...

OBJECTIVE We sought to evaluate clinical correlates of low serum carnitine levels in hospitalized psychiatric patients. METHODS We retrospectively reviewed the charts of 40 psychiatric inpatients identified to have low serum carnitine levels. RESULTS Cognitive impairment was present in 38 (95%) cases, frequently accompanied by imbalance, agitation and extrapyramidal symptoms. Valproate ther...

Introduction A trimethylated amino acid, roughly similar in structure to choline, L-carnitine is a cofactor required for transformation of free long-chain fatty acids into acylcarnitines, and for their subsequent transport into the mitochondrial matrix, where they undergo beta-oxidation for cellular energy production. Conditions that appear to benefit from exogenous supplementation of L-carniti...

We report the crystal structure of a binary complex of human peroxisomal carnitine acetyltransferase and the substrate l-carnitine, refined to a resolution of 1.8 Angstrom with an R(factor) value of 18.9% (R(free)=22.3%). L-carnitine binds to a preformed pocket in the active site tunnel of carnitine acetyltransferase aligned with His(322). The quaternary nitrogen of carnitine forms a pi-cation ...

Carnitine deficiency has been demonstrated in diabetic hearts, and it is also well known that L-carnitine administration has a beneficial effect on cardiac function. Carnitine treatment would be expected to reduce the accumulation of long-chain acylcarnitine. However, many reports have shown that myocardial long-chain acylcarnitine levels were increased following treatment with L-carnitine in w...