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A Mouse Model of L-2-Hydroxyglutaric Aciduria, a Disorder of Metabolite Repair
The purpose of the present work was to progress in our understanding of the pathophysiology of L-2-hydroxyglutaric aciduria, due to a defect in L-2-hydroxyglutarate dehydrogenase, by creating and studying a mouse model of this disease. L-2-hydroxyglutarate dehydrogenase-deficient mice (l2hgdh (-/-))...
Autores principales: | Rzem, Rim, Achouri, Younes, Marbaix, Etienne, Schakman, Olivier, Wiame, Elsa, Marie, Sandrine, Gailly, Philippe, Vincent, Marie-Françoise, Veiga-da-Cunha, Maria, Van Schaftingen, Emile |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4357467/ https://www.ncbi.nlm.nih.gov/pubmed/25763823 http://dx.doi.org/10.1371/journal.pone.0119540 |
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