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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Detalhes bibliográficos
Publicado no:Dis Model Mech
Main Authors: Tsai, Hui-Ying, Wu, Shih-Cheng, Li, Jian-Chiuan, Chen, Yu-Min, Chan, Chih-Chiang, Chen, Chun-Hong
Formato: Artigo
Idioma:Inglês
Publicado em: The Company of Biologists Ltd 2020
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7473638/
https://ncbi.nlm.nih.gov/pubmed/32680850
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.044750
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