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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...

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Detalles Bibliográficos
Autores principales: Fu, Xi, Gao, Hongjie, Tian, Fengyan, Gao, Jinzhi, Lou, Liping, Liang, Yan, Ning, Qin, Luo, Xiaoping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
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