L-Carnitine Improves Skeletal Muscle Fat Oxidation in Primary Carnitine Deficiency
نویسندگان
چکیده
منابع مشابه
Malonyl-CoA and carnitine in regulation of fat oxidation in human skeletal muscle during exercise.
Intracellular mechanisms regulating fat oxidation were investigated in human skeletal muscle during exercise. Eight young, healthy, moderately trained men performed bicycle exercise (60 min, 65% peak O2 consumption) on two occasions, where they ingested either 1) a high-carbohydrate diet (H-CHO) or 2) a low-carbohydrate diet (L-CHO) before exercise to alter muscle glycogen content as well as to...
متن کاملAbnormal sodium stimulation of carnitine transport in primary carnitine deficiency.
Primary carnitine deficiency is an autosomal recessive disorder of fatty acid oxidation characterized by hypoketotic hypoglycemia and skeletal and cardiac myopathy. It is caused by mutations in the sodium-dependent carnitine cotransporter OCTN2. The majority of natural mutations identified in this and other Na(+)/solute symporters introduce premature termination codons or impair insertion of th...
متن کاملPrimary Carnitine Deficiency and Cardiomyopathy
Carnitine is essential for the transfer of long-chain fatty acids from the cytosol into mitochondria for subsequent β-oxidation. A lack of carnitine results in impaired energy production from long-chain fatty acids, especially during periods of fasting or stress. Primary carnitine deficiency (PCD) is an autosomal recessive disorder of mitochondrial β-oxidation resulting from defective carnitine...
متن کاملMR in children with L-carnitine deficiency.
PURPOSE To describe the MR imaging findings in five children with proved L-carnitine deficiency. METHODS MR imaging studies (five without contrast, two with contrast) were obtained in five children (mean age, 9 years) who presented with stroke symptoms and who proved to have L-carnitine deficiency as established by serum levels. RESULTS In three of five patients, infarctions were confined t...
متن کاملRethinking the regulation of l-carnitine transport in skeletal muscle cells. Focus on "Multiple AMPK activators inhibit l-carnitine uptake in C2C12 skeletal muscle myotubes".
CARNITINE IS A CRITICAL COFACTOR in the metabolism of lipids and therefore in the production of cellular energy. L-Carnitine, the active form, plays an important role in oxidizing fatty acids, transporting long chain fatty acids across mitochondrial membrane, and modulating intracellular coenzyme A homeostasis (3). L-Carnitine uptake into cells is mediated primarily by the organic cation/carnit...
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ژورنال
عنوان ژورنال: The Journal of Clinical Endocrinology & Metabolism
سال: 2018
ISSN: 0021-972X,1945-7197
DOI: 10.1210/jc.2018-00953