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particolarmente importante per chi fa esercizio fisico, in periodi di digiuno o di ridotto apporto calorico, perch aiuta a convertire i grassi in energia e favorisce il metabolismo degli aminoacidi a catena ramificata come valina, leucina e isoleucina

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Propionic, methylmalonic and isovaleric acidurias are inborn errors of metabolism characterized by the accumulation of the respective organic acids due to a deficiency of the enzymes propionyl coenzyme A carboxylase, methylmalonyl coenzyme A mutase or isovaleryl coenzyme A dehydrogenase, respectively

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