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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Dettagli Bibliografici
Pubblicato in:Redox Biol
Autori principali: Torres, Sandra, Matías, Nuria, Baulies, Anna, Nuñez, Susana, Alarcon-Vila, Cristina, Martinez, Laura, Nuño, Natalia, Fernandez, Anna, Caballeria, Joan, Levade, Thierry, Gonzalez-Franquesa, Alba, Garcia-Rovés, Pablo, Balboa, Elisa, Zanlungo, Silvana, Fabrías, Gemma, Casas, Josefina, Enrich, Carlos, Garcia-Ruiz, Carmen, Fernández-Checa, José C.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Elsevier 2016
Soggetti:
Research Paper
Accesso online: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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