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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...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473638/ https://www.ncbi.nlm.nih.gov/pubmed/32680850 http://dx.doi.org/10.1242/dmm.044750 |