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y acumulacin intracelular de amonio en forma similar a lo que ocurre en el dficit de la enzima CPS I

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The latter can be reduced by thioredoxin (Trx) 2, which is further reduced by thioredoxin reductase [28]

284 Thus, successful implementation of the KD requires well-organized and consistently functioning cooperation between caregivers and patients, and clinical settings that support this cooperation