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

• Hoofdauteurs: Hui-Ying Tsai (Auteur), Shih-Cheng Wu (Auteur), Jian-Chiuan Li (Auteur), Yu-Min Chen (Auteur), Chih-Chiang Chan (Auteur), Chun-Hong Chen (Auteur)
• Formaat: Boek
• Gepubliceerd in: The Company of Biologists, 2020-08-01T00:00:00Z.
• Onderwerpen: article
• Online toegang: 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...