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Loss of the Drosophila branched-chain α-ketoacid dehydrogenase complex results in neuronal dysfunction

Maple syrup urine disease (MSUD) is an inherited error in the metabolism of branched-chain amino acids (BCAAs) caused by a severe deficiency of the branched-chain α-ketoacid dehydrogenase (BCKDH) complex, which ultimately leads to neurological disorders. The limited therapies, including protein-rest...

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Detalles Bibliográficos
Autores principales: Tsai, Hui-Ying, Wu, Shih-Cheng, Li, Jian-Chiuan, Chen, Yu-Min, Chan, Chih-Chiang, Chen, Chun-Hong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473638/
https://www.ncbi.nlm.nih.gov/pubmed/32680850
http://dx.doi.org/10.1242/dmm.044750