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Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann–Pick disease, type C1

Niemann–Pick disease, type C1 (NPC1), which arises from a mutation in the NPC1 gene, is characterized by abnormal cellular storage and transport of cholesterol and other lipids that leads to hepatic disease and progressive neurological impairment. Oxidative stress has been hypothesized to contribute...

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Autors principals: Fu, Rao, Wassif, Christopher A., Yanjanin, Nicole M., Watkins-Chow, Dawn E., Baxter, Laura L., Incao, Art, Liscum, Laura, Sidhu, Rohini, Firnkes, Sally, Graham, Mark, Ory, Daniel S., Porter, Forbes D., Pavan, William J.
Format: Artigo
Idioma:Inglês
Publicat: Oxford University Press 2013
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3736870/
https://ncbi.nlm.nih.gov/pubmed/23666527
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddt206
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