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A diagnosis of primary carnitine deficiency requires that serum, red cell and/or tissue carnitine levels be low and that the patient does not have a primary defect in fatty acid or organic acid oxidation (see CLINICAL PHARMACOLOGY )

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The inhibitors of intracellular Gln transport, such as L--Glutamyl-p-nitroanilide (GPNA) and its derivative V-9032, were ineffective in reducing Gln concentration and are characterized by a lack of specificity [147,148,149]

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Es kommt in allen Krperzellen vor, mit besonders hohen Konzentrationen in der Leber