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

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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Bibliographic Details
Main Authors: Hui-Ying Tsai, Shih-Cheng Wu, Jian-Chiuan Li, Yu-Min Chen, Chih-Chiang Chan, Chun-Hong Chen
Format: Article
Language:English
Published: The Company of Biologists 2020-08-01
Series:Disease Models & Mechanisms
Subjects:
msud
neuronal apoptosis
oxidative stress
drosophila
Online Access:http://dmm.biologists.org/content/13/8/dmm044750

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