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Article

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

Disease models & mechanisms - 27 Aug 2020

Tsai Hui-Ying, Wu Shih-Cheng, Li Jian-Chiuan, Chen Yu-Min, Chan Chih-Chiang, Chen Chun-Hong

Abstract excerpt

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-restricted diets and liver transplants, are not as effective as they could be for the treatment of MSUD due to the...

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Share to Amino Acids, Branched-Chain
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 32680850
DOI: 10.1242/dmm.044750
PMCID: PMC7473638

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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