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15 DIFFERENTIALLY IMPAIRED OXIDATION OF BRANCHED-CHAIN AMINO ACIDS IN MAPLE SYRUP URINE DISEASE (MSUD)
Whether the impaired branched-chain 2-oxo acid dehydrogenase (BCOA-DH) activity in MSUD is concurrently or rather differentially reduced against the different 2-oxo acid substrates was assessed by measuring the 1...