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71 In addition, extra-mitochondrial localization of branched-chain -keto acid dehydrogenase (BCKDH), a rate-limiting enzyme in BCAAs metabolism, has been reported in type 2 diabetic rat model (OLETF)

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demonstrated, in neurons differentiated from iPSCs derived from MSA patients, the existing genotypephenotype causal relationship between COQ2 mutations and the mitochondrial respiratory dysfunction observed in the disease

Std: standard feed, Gln: glutamine-supplemented feed, GSH: glutathione-supplemented feed