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Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease
Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulatio...
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| Publicado en: | Redox Biol |
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| Main Authors: | , , , , , , , , , , , , , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado: |
Elsevier
2016
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| Assuntos: | |
| Acceso en liña: | https://ncbi.nlm.nih.gov/pmc/articles/PMC5123076/ https://ncbi.nlm.nih.gov/pubmed/27888692 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.redox.2016.11.010 |
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