Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease

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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Bibliographic Details
Main Authors: Sandra Torres, Nuria Matías, Anna Baulies, Susana Nuñez, Cristina Alarcon-Vila, Laura Martinez, Natalia Nuño, Anna Fernandez, Joan Caballeria, Thierry Levade, Alba Gonzalez-Franquesa, Pablo Garcia-Rovés, Elisa Balboa, Silvana Zanlungo, Gemma Fabrías, Josefina Casas, Carlos Enrich, Carmen Garcia-Ruiz, José C. Fernández-Checa
Format: Article
Language:English
Published: Elsevier 2017-04-01
Series:Redox Biology
Online Access:http://www.sciencedirect.com/science/article/pii/S2213231716302567

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http://www.sciencedirect.com/science/article/pii/S2213231716302567

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