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Pre-Symptomatic Activation of Antioxidant Responses and Alterations in Glucose and Pyruvate Metabolism in Niemann-Pick Type C1-Deficient Murine Brain

Niemann-Pick Type C (NPC) disease is an autosomal recessive neurodegenerative disorder caused in most cases by mutations in the NPC1 gene. NPC1-deficiency is characterized by late endosomal accumulation of cholesterol, impaired cholesterol homeostasis, and a broad range of other cellular abnormaliti...

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Detalles Bibliográficos
Autores principales: Kennedy, Barry E., LeBlanc, Veronique G., Mailman, Tiffany M., Fice, Debra, Burton, Ian, Karakach, Tobias K., Karten, Barbara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3867386/
https://www.ncbi.nlm.nih.gov/pubmed/24367541
http://dx.doi.org/10.1371/journal.pone.0082685