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Brain damage in methylmalonic aciduria: 2-methylcitrate induces cerebral ammonium accumulation and apoptosis in 3D organotypic brain cell cultures

BACKGROUND: Methylmalonic aciduria is an inborn error of metabolism characterized by accumulation of methylmalonate (MMA), propionate and 2-methylcitrate (2-MCA) in body fluids. Early diagnosis and current treatment strategies aimed at limiting the production of these metabolites are only partially...

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Detalles Bibliográficos
Autores principales:	Jafari, Paris, Braissant, Olivier, Zavadakova, Petra, Henry, Hugues, Bonafé, Luisa, Ballhausen, Diana
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2013
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3567978/ https://www.ncbi.nlm.nih.gov/pubmed/23298464 http://dx.doi.org/10.1186/1750-1172-8-4

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