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Effect of the branched-chain α-keto acids accumulating in maple syrup urine disease on S100B release from glial cells

✍ Scribed by Cláudia Funchal; Francine Tramontina; André Quincozes dos Santos; Daniela Fraga de Souza; Carlos Alberto Gonçalves; Regina Pessoa-Pureur; Moacir Wajner

Book ID
119302315
Publisher
Elsevier Science
Year
2007
Tongue
English
Weight
574 KB
Volume
260
Category
Article
ISSN
0022-510X

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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