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

• Prif Awduron: Hui-Ying Tsai (Awdur), Shih-Cheng Wu (Awdur), Jian-Chiuan Li (Awdur), Yu-Min Chen (Awdur), Chih-Chiang Chan (Awdur), Chun-Hong Chen (Awdur)
• Fformat: Llyfr
• Cyhoeddwyd: The Company of Biologists, 2020-08-01T00:00:00Z.
• Pynciau: article
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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...