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

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

<p>Abstract</p> <p>Background</p> <p>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...

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Bibliographic Details
Main Authors: Jafari Paris, Braissant Olivier, Zavadakova Petra, Henry Hugues, Bonafé Luisa, Ballhausen Diana
Format: Article
Language:English
Published: BMC 2013-01-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Methylmalonic aciduria
Methylmalonate
2-methylcitrate
Propionate
Hyperammonemia
Apoptosis
Brain damage
Neurotoxicity
Online Access:http://www.ojrd.com/content/8/1/4

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