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It is assumed that the level of acylcarnitines in blood mirrors the accumulating acyl-CoA species inside mitochondria which cause the carnitine shuttle to operate in the reverse fashion [32]

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182 Coenzyme Q10 Coenzyme Q10 (CoQ10) is centrally involved in cellular energy production in the mitochondria and has been studied for the treatment of cardiovascular disease

It is important to note that AGAT deficiency is the only disorder that presents with additional myopathy, which may be related to guanidinoacetic acid (GAA) deficiency

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Sin embargo, el proceso presentaba problemas como el uso de sustancias txicas que contienen cianuro, el alto costo de los agentes de resolucin y los catalizadores, la dificultad para aprovechar la otra mitad del enantimero separado, los numerosos pasos de reaccin, el bajo rendimiento, los altos costos y el elevado precio del producto