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For example, average quercetin consumption has been reported at 18 mg in China, 16.2 mg in Japan, 18.48 mg in Spain, and 9.75 mg in the United States ( 3.1.2 Bioavailability and pharmacokinetics of quercetin The burgeoning interest in quercetins health benefits has catalyzed the production of nutraceuticals containing high doses of this metabolites (Wagner et al., 2006)

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Thus, while these studies provide valuable information about the BCATi and metabolic differences between healthy and MMUT-deficient hepatocytes, they do not allow us to test the idea that BCAT inhibition in other organs, namely the skeletal muscle, could lower toxic catabolites in the liver and be useful for the therapy of MMA

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