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

• Main Authors: Hui-Ying Tsai (Author), Shih-Cheng Wu (Author), Jian-Chiuan Li (Author), Yu-Min Chen (Author), Chih-Chiang Chan (Author), Chun-Hong Chen (Author)
• Format: Book
• Published: The Company of Biologists, 2020-08-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...