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

Redox Biology

Volumn 11, Issue , 2017, Pages 60-72

  Torres, Sandra ^a,b   Matías, Nuria ^a,b   Baulies, Anna ^a,b   Nuñez, Susana ^a,b   Alarcon Vila, Cristina ^a,b   Martinez, Laura ^a,b   Nuño, Natalia ^a,b   Fernandez, Anna ^a,b   Caballeria, Joan ^b   Levade, Thierry ^c   Gonzalez Franquesa, Alba ^b   Garcia Rovés, Pablo ^b   Balboa, Elisa ^d   Zanlungo, Silvana ^d   Fabrías, Gemma ^e   Casas, Josefina ^e   Enrich, Carlos ^b,f   Garcia Ruiz, Carmen ^a,b,g   Fernández Checa, José C ^a,b,g  

^a INST D INVESTIGACIONS B   (Spain)

^b INSTITUT D INVESTIGACIONS BIOMÈDIQUES AUGUST PI I SUNYER IDIBAPS   (Spain)

^c INSERM   (France)

^d PONTIFICIA UNIVERSIDAD CATÓLICA DE CHILE   (Chile)

^e DEPARTMENT OF ENVIRONMENTAL CHEMISTRY   (Spain)

^f UNIVERSITY OF BARCELONA   (Spain)

^g UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

Author keywords

Ceramide; Cerebellum; Hepatosplenomegaly; Lysosomal disorders; Sphingolipids, Mitochondrial GSH

Indexed keywords

ACETYLCYSTEINE; CALBINDIN; GLUTATHIONE; GLUTATHIONE ETHYL ESTER; SPHINGOLIPID; CHOLESTEROL; NPC1 PROTEIN, MOUSE; NPC2 PROTEIN, MOUSE; PROTEIN; VESICULAR TRANSPORT PROTEIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN LEVEL; CELL DEATH; CEREBELLUM; CONTROLLED STUDY; FIBROBLAST; HUMAN; HUMAN CELL; LIFESPAN; LIPID BRAIN LEVEL; LIPID LIVER LEVEL; LIPIDOMICS; LIVER LEVEL; LOCOMOTION; MITOCHONDRIAL ENERGY TRANSFER; MITOCHONDRIAL RESPIRATION; MITOCHONDRION; MOUSE; NIEMANN PICK DISEASE; NONHUMAN; OXIDATIVE PHOSPHORYLATION; OXIDATIVE STRESS; PRIORITY JOURNAL; PURKINJE CELL; RESPIROMETRY; SURVIVAL RATE; TREATMENT OUTCOME; ANIMAL; GENETICS; KNOCKOUT MOUSE; LYSOSOME; METABOLISM; MUTATION; PATHOLOGY;

ACETYLCYSTEINE; ANIMALS; CEREBELLUM; CHOLESTEROL; GLUTATHIONE; HUMANS; LYSOSOMES; MICE; MICE, KNOCKOUT; MITOCHONDRIA; MUTATION; NIEMANN-PICK DISEASE, TYPE C; OXIDATIVE PHOSPHORYLATION; PROTEINS; PURKINJE CELLS; VESICULAR TRANSPORT PROTEINS;

EID: 84996844004     PISSN: 22132317     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.redox.2016.11.010     Document Type: Article

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