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Reduction of Glutamate Uptake into Cerebral Cortex of Developing Rats by the Branched-Chain Alpha-Keto Acids Accumulating in Maple Syrup Urine Disease

✍ Scribed by Cláudia Funchal; AlineMeyer Rosa; Moacir Wajner; Susana Wofchuk; ReginaPessoa Pureur

Book ID
111602248
Publisher
Springer
Year
2004
Tongue
English
Weight
71 KB
Volume
29
Category
Article
ISSN
0364-3190

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Evidence that the branched-chain α-keto
Evidence that the branched-chain α-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex
✍ Cláudia Funchal; Carmem Gottfried; Lúcia Maria Vieira De Almeida; Moacir Wajner; 📂 Article 📅 2004 🏛 John Wiley and Sons 🌐 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