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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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Detalles Bibliográficos
Autores principales:	Xu, Jing, Jakher, Youseff, Ahrens-Nicklas, Rebecca C.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2020
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7590055/ https://www.ncbi.nlm.nih.gov/pubmed/33050626 http://dx.doi.org/10.3390/ijms21207490

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