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Ammonium Accumulation and Cell Death in a Rat 3D Brain Cell Model of Glutaric Aciduria Type I
Glutaric aciduria type I (glutaryl-CoA dehydrogenase deficiency) is an inborn error of metabolism that usually manifests in infancy by an acute encephalopathic crisis and often results in permanent motor handicap. Biochemical hallmarks of this disease are elevated levels of glutarate and 3-hydroxygl...
Autores principales: | Jafari, Paris, Braissant, Olivier, Zavadakova, Petra, Henry, Hugues, Bonafé, Luisa, Ballhausen, Diana |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3542363/ https://www.ncbi.nlm.nih.gov/pubmed/23326493 http://dx.doi.org/10.1371/journal.pone.0053735 |
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