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Finally, considering that mitochondria emerged as the most sensitive organelle to Cu toxicity in Wilson disease 13,31 , we investigated the impact of PrP suppression on mitochondrial function

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Evidence for a role of the 2-oxoglutarate carrier as a GSH transporter was based on substrate specificity and pattern of inhibition in rabbit kidney mitochondria and in a partially purified preparation of mitochondrial transporters from kidney mitoplasts reconstituted in proteoliposomes (20, 21)