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carnitine deficiency

   Also found in: Dictionary/thesaurus, Acronyms, Encyclopedia, Wikipedia 0.03 sec.
carnitine
coenzyme of fatty acid oxidation and acetyl transfer; often designated vitamin BT, due to its vitamin role in Tenebrio sp. Present in high concentrations (5% dry weight) in meat extracts.

carnitine acetyltransferase
enzyme associated with buffering of acetyl groups from acetyl CoA.
carnitine deficiency
associated with myocardial disease in dogs, particularly Boxers.

carnitine deficiency
Metabolic disease A condition caused by ↓ carnitine palmityl transferase, failure of carnitine transport in kidney, muscle, fibroblasts or cobalamin deficiency, electron transfer flavoprotein deficiency, Fanconi syndrome, isovaleric acidemia, medium chain acylCoA dehydrogenase deficiency, methylmalonic and propionic acidemias; Pts receiving valproate for seizure disorders may develop a toxicity syndrome with associated carnitine deficiency Clinical Myoglobinuria, renal failure, hypoglycemia, hypotonia, hepatomegaly, hepatic coma, CHF, neurologic changes–progressive myasthenia, encephalopathy, lethargy, coma, death, cardiomegaly, cardiac arrest, impaired growth and development


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Kinetic compartmental analysis of carnitine metabolism in the human carnitine deficiency syndromes: evidence for alterations in tissue carnitine transport.
The accumulation of the last two metabolites above a toxic threshold, as well as the secondary carnitine deficiency that develops, produces the clinical syndrome of isovaleric acidemia (Fig.
Sigma-Tau Pharmaceuticals' products include Carnitor(R), an FDA-approved treatment for carnitine deficiency in dialysis patients and patients with
 
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