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MSUD is an autosomal recessive disorder that results from a deficiency in the enzyme, branched-chain -keto acid dehydrogenase (BCKD), that is involved in the catabolism of the branched-chain amino acids (BCAA), leucine, isoleucine, and valine

J Neurosci 33(24):99579962 Kathrin, Tllner Saskia, Wolf Wolfgang, Lscher Manuela, Gernert (2011) The anticonvulsant response to valproate in kindled rats is correlated with its effect on neuronal firing in the substantia nigra pars reticulata: a new mechanism of pharmacoresistance

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