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Inhibition of Brain Energy Metabolism by the Branched-chain Amino Acids Accumulating in Maple Syrup Urine Disease

✍ Scribed by César A. Ribeiro; Ângela M. Sgaravatti; Rafael B. Rosa; Patrícia F. Schuck; Vanessa Grando; Anna L. Schmidt; Gustavo C. Ferreira; Marcos L. S. Perry; Carlos S. Dutra-Filho; Moacir Wajner


Publisher
Springer
Year
2007
Tongue
English
Weight
442 KB
Volume
33
Category
Article
ISSN
0364-3190

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Maple syrup urine disease: Branched-chai
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## 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

Quantification of branched-chain amino a
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## Abstract Individuals with maple syrup urine disease (MSUD) have an inherited metabolic disorder resulting in a deficiency in the branched‐chain keto‐acid dehydrogenase complex. As a result, these individuals have elevated concentrations of the branched‐chain amino acids valine, isoluecine, allo‐