Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders

Maple syrup urine disease (MSUD) is an autosomal recessive disorder caused by decreased activity of the branched-chain α-ketoacid dehydrogenase complex (BCKDC), which catalyzes the irreversible catabolism of branched-chain amino acids (BCAAs). Current management of this BCAA dyshomeostasis consists...

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Publicado en:Int J Mol Sci
Autores principales: Xu, Jing, Jakher, Youseff, Ahrens-Nicklas, Rebecca C.
Formato: Artigo
Lenguaje:Inglês
Publicado: MDPI 2020
Materias:
Review
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC7590055/
https://ncbi.nlm.nih.gov/pubmed/33050626
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21207490
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