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Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia?

BACKGROUND: Hyperammonemia in patients with methylmalonic aciduria (MMA) and propionic aciduria (PA) is caused by accumulation of propionyl-CoA which decreases the synthesis of N-acetyl-glutamate, the natural activator of carbamyl phosphate synthetase 1. A treatment approach with carglumic acid, the...

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Detalles Bibliográficos
Autores principales: Levrat, Virginie, Forest, Isabelle, Fouilhoux, Alain, Acquaviva, Cécile, Vianey-Saban, Christine, Guffon, Nathalie
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2262878/
https://www.ncbi.nlm.nih.gov/pubmed/18234091
http://dx.doi.org/10.1186/1750-1172-3-2

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