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Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and dysmyelination in affected individuals. Treatment requires life-long dietary restriction and monitoring of branched-chain amino acids to avoid brain...

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Detalles Bibliográficos
Autores principales: Zinnanti, William J., Lazovic, Jelena, Griffin, Kathleen, Skvorak, Kristen J., Paul, Harbhajan S., Homanics, Gregg E., Bewley, Maria C., Cheng, Keith C., LaNoue, Kathryn F., Flanagan, John M.
Formato: Texto
Lenguaje:English
Publicado: Oxford University Press 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2668944/
https://www.ncbi.nlm.nih.gov/pubmed/19293241
http://dx.doi.org/10.1093/brain/awp024