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Listar por autor "Mata, Mario de la"
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Artículo
Coenzyme Q(10) Therapy
Garrido-Maraver, Juan; Cordero, Mario D.; Oropesa-Ávila, Manuel; Fernández Vega, Alejandro; Mata, Mario de la; Delgado Pavón, Ana; Miguel Rodríguez, Manuel de; Sánchez-Alcázar, José Antonio (KARGER, 2014)For a number of years, coenzyme Q 10 (CoQ 10 ) was known for its key role in mitochondrial bioenergetics; later ...
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Artículo
Fluorinated chaperone-ß-cyclodextrin formulations for ß-glucocerebrosidase activity enhancement in neuronopathic Gaucher disease
García Moreno, M. Isabel; Mata, Mario de la; Sánchez Fernández, Elena Matilde; Benito, Juan M.; Díaz Quintana, Antonio Jesús; Fustero, Santos; Nanba, Eiji; Higaki, Katsumi; Sánchez-Alcazar, José Antonio; García Fernández, José Manuel; Ortiz Mellet, Carmen (American Chemical Society, 2017)Amphiphilic glycomimetics encompassing a rigid, undistortable nor-tropane skeleton based on 1,6-anhydro-L-idonojirimycin ...
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Artículo
Pantothenate Rescues Iron Accumulation in Pantothenate Kinase-Associated Neurodegeneration Depending on the Type of Mutation
Álvarez Córdoba, Mónica; Fernández Khoury, Aida; Villanueva Paz, Marina; Gómez Navarro, Carmen; Villalón García, Irene; Suárez Rivero, Juan M.; Povea Cabello, Suleva; Mata, Mario de la; Cotán, David; Talaverón Rey, Marta; Pérez Pulido, Antonio J.; Salas, Joaquín J.; Pérez Villegas, Eva Mª; Díaz Quintana, Antonio Jesús; Armengol, José A.; Sánchez Alcázar, José A. (Springer Nature, 2019)Neurodegeneration with brain iron accumulation (NBIA) is a group of inherited neurologic disorders in which iron accumulates ...
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Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease
Mata, Mario de la; Cotán, David; Oropesa Ávila, Manuel; Garrido Maraver, Juan; Cordero Morales, Mario David; Ortiz Mellet, Carmen; Ybot González, Patricia; García Fernández, José Manuel (Nature Publishing Group, 2015)Gaucher disease (GD) is caused by mutations in the GBA1 gene, which encodes lysosomal β-glucocerebrosidase. Homozygosity ...