Evidence that the branched-chain α-keto
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Cláudia Funchal; Carmem Gottfried; Lúcia Maria Vieira De Almeida; Moacir Wajner;
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Article
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2004
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John Wiley and Sons
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English
⚖ 609 KB
## Abstract Severe neurological symptoms, cerebral edema, and atrophy are common features of the inherited metabolic disorder maple syrup urine disease (MSUD). However, the pathomechanisms involved in the neuropathology of this disease are not well established. In this study, we investigated the ef