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Related to Carnitine deficiency: L-carnitine


C7H15NO3α-Amino-β-hydroxybutyric acid trimethylbetaine; a constituent of striated muscle and liver, identical with vitamin B T.



betaine-γ-amino-β-oxybutyric acid, (CH3)3NCH2CH(OH)CH2CO2, a crystalline compound with basic properties; dissolves readily in water and alcohol. Its molecular mass is 161.21, and its melting point, 195°-197°C (with decomposition).

Carnitine is primarily found in animal muscle, from which it was first extracted by V. S. Gulevich (1905); it is also found in bacteria and plants. It takes part in fatty exchange within an organism by acting as a carrier of fatty acid radicals through the membranes of the mitochondria. These membranes are impermeable to activated fatty acids (compounds with coenzyme A). With the aid of carnitine, therefore, fatty acids enter the scope of activity of the oxidizing enzymes localized within the mitochondria. Carnitine apparently also participates in the reverse transport of fatty acids. It is an essential dietary constituent and a growth factor in certain insects; therefore it is considered to be a vitamin (vitamin BT).

References in periodicals archive ?
And not every patient I've treated for carnitine deficiency has shown a dramatic response.
Primary systemic carnitine deficiency is a very rare genetic disorder that typically presents in infants.
A deficiency of C2 coupled with low FC increases the probability of a primary or secondary carnitine deficiency.
These researchers consider the carnitine deficiency may be due in part to intramitochondrial accumulation of acylcarnitines, followed by renal excretion.
Secondary carnitine deficiency is likely to occur, requiring supplementation.
This is thought to be in part related to massive oxidative stress induced by kidney disease, and partly to inadequate energy management in cardiac tissues induced by carnitine deficiency.
A decrease in muscular carnitine could be associated with a loss of muscle strength, although the definition of a carnitine deficiency is not fulfilled.
Speakers at the conference, "Carnitine: the Science Behind a Conditionally Essential Nutrient," will discuss the scientific body of knowledge that suggests that carnitine deficiency may be associated with a host of chronic diseases, including cardiovascular and end- stage renal disease, cancer, male infertility -- even aging.
A partial list of some of the most common hereditary disorders includes medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (1015), very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (2), long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency (16), and systemic carnitine deficiency (2, 3,17,18).
In particular, it sought to identify risk factors that might help anticipate carnitine deficiency and to predict the response to treatment.
Spectracef is contraindicated in patients with known allergy to the cephalosporin class of antibiotics or any of its components, and in patients with carnitine deficiency or inborn errors of metabolism that may result in clinically significant carnitine deficiency.
Measurements of free and total carnitine in plasma are important in the diagnosis and clinical management of patients with carnitine deficiency syndromes and certain inborn errors of metabolism (1).
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