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Mechanistic Effects of Amino Acids and Glucose in a Novel Glutaric Aciduria Type 1 Cell Model
Acute neurological crises involving striatal degeneration induced by a deficiency of glutaryl-CoA dehydrogenase (GCDH) and the accumulation of glutaric (GA) and 3-hydroxyglutaric acid (3-OHGA) are considered to be the most striking features of glutaric aciduria type I (GA1). In the present study, we...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4198201/ https://www.ncbi.nlm.nih.gov/pubmed/25333616 http://dx.doi.org/10.1371/journal.pone.0110181 |