Search Results - "Alcázar, José A."

Dynamic Reorganization of the Cytoskeleton during Apoptosis: The Two Coffins Hypothesis by Povea-Cabello, Suleva, Oropesa-Ávila, Manuel, de la Cruz-Ojeda, Patricia, Villanueva-Paz, Marina, de la Mata, Mario, Suárez-Rivero, Juan Miguel, Álvarez-Córdoba, Mónica, Villalón-García, Irene, Cotán, David, Ybot-González, Patricia, Sánchez-Alcázar, José A

“…During apoptosis, cells undergo characteristic morphological changes in which the cytoskeleton plays an active role. The cytoskeleton rearrangements have been…”
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Vitamin E prevents lipid peroxidation and iron accumulation in PLA2G6-Associated Neurodegeneration by Villalón-García, Irene, Álvarez-Córdoba, Mónica, Povea-Cabello, Suleva, Talaverón-Rey, Marta, Villanueva-Paz, Marina, Luzón-Hidalgo, Raquel, Suárez-Rivero, Juan M., Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Salas, Joaquín J., Falcón-Moya, Rafael, Rodríguez-Moreno, Antonio, Armengol, José A., Sánchez-Alcázar, José A.

“…PLA2G6-Associated Neurodegeneration (PLAN) is a rare neurodegenerative disease with autosomal recessive inheritance, which belongs to the NBIA…”
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Parkin-mediated mitophagy and autophagy flux disruption in cellular models of MERRF syndrome by Villanueva-Paz, Marina, Povea-Cabello, Suleva, Villalón-García, Irene, Álvarez-Córdoba, Mónica, Suárez-Rivero, Juan M., Talaverón-Rey, Marta, Jackson, Sandra, Falcón-Moya, Rafael, Rodríguez-Moreno, Antonio, Sánchez-Alcázar, José A.

“…Mitochondrial diseases are considered rare genetic disorders characterized by defects in oxidative phosphorylation (OXPHOS). They can be provoked by mutations…”
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Pathophysiological characterization of MERRF patient-specific induced neurons generated by direct reprogramming by Villanueva-Paz, Marina, Povea-Cabello, Suleva, Villalón-García, Irene, Suárez-Rivero, Juan M., Álvarez-Córdoba, Mónica, de la Mata, Mario, Talaverón-Rey, Marta, Jackson, Sandra, Sánchez-Alcázar, José A.

“…Mitochondrial diseases are a group of rare heterogeneous genetic disorders caused by total or partial mitochondrial dysfunction. They can be caused by…”
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Fluorinated Chaperone−β-Cyclodextrin Formulations for β‑Glucocerebrosidase Activity Enhancement in Neuronopathic Gaucher Disease by García-Moreno, M. Isabel, de la Mata, Mario, Sánchez-Fernández, Elena M, Benito, Juan M, Díaz-Quintana, Antonio, Fustero, Santos, Nanba, Eiji, Higaki, Katsumi, Sánchez-Alcázar, José A, García Fernández, José M, Ortiz Mellet, Carmen

“…Amphiphilic glycomimetics encompassing a rigid, undistortable nortropane skeleton based on 1,6-anhydro-l-idonojirimycin and a polyfluorinated antenna, when…”
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Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease by de la Mata, Mario, Cotán, David, Oropesa-Ávila, Manuel, Garrido-Maraver, Juan, Cordero, Mario D., Villanueva Paz, Marina, Delgado Pavón, Ana, Alcocer-Gómez, Elizabet, de Lavera, Isabel, Ybot-González, Patricia, Paula Zaderenko, Ana, Ortiz Mellet, Carmen, Fernández, José M. García, Sánchez-Alcázar, José A.

“…Gaucher disease (GD) is caused by mutations in the GBA1 gene, which encodes lysosomal β-glucocerebrosidase. Homozygosity for the L444P mutation in GBA1 is…”
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Screening of effective pharmacological treatments for MELAS syndrome using yeasts, fibroblasts and cybrid models of the disease by Garrido‐Maraver, Juan, Cordero, Mario D, Moñino, Irene Domínguez, Pereira‐Arenas, Sheila, Lechuga‐Vieco, Ana V, Cotán, David, De la Mata, Mario, Oropesa‐Ávila, Manuel, De Miguel, Manuel, Bautista Lorite, Juan, Rivas Infante, Eloy, Álvarez‐Dolado, Manuel, Navas, Plácido, Jackson, Sandra, Francisci, Silvia, Sánchez‐Alcázar, José A

“…BACKGROUND AND PURPOSE MELAS (mitochondrial encephalomyopathy, lactic acidosis and stroke‐like episodes) is a mitochondrial disease most usually caused by…”
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Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels by Ãlvarez-Córdoba, Mónica, Reche-López, Diana, Cilleros-Holgado, Paula, Talaverón-Rey, Marta, Villalón-García, Irene, Povea-Cabello, Suleva, Suárez-Rivero, Juan M, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Piéero-Pérez, Rocío, Sánchez-Alcázar, José A

“…Neurodegeneration with brain iron accumulation (NBIA) is a group of rare neurogenetic disorders frequently associated with iron accumulation in the basal…”
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Vicious cycle of lipid peroxidation and iron accumulation in neurodegeneration by Villalón-García, Irene, Povea-Cabello, Suleva, Álvarez-Córdoba, Mónica, Talaverón-Rey, Marta, Suárez-Rivero, Juan, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Reche-López, Diana, Cilleros-Holgado, Paula, Piñero-Pérez, Rocío, Sánchez-Alcázar, José

“…Lipid peroxidation and iron accumulation are closely associated with neurodegenerative diseases, such as Alzheimer's, Parkinson's, and Huntington's diseases,…”
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Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis by López-Martín, José M., Salviati, Leonardo, Trevisson, Eva, Montini, Giovanni, DiMauro, Salvatore, Quinzii, Catarina, Hirano, Michio, Rodriguez-Hernandez, Angeles, Cordero, Mario D., Sánchez-Alcázar, José A., Santos-Ocaña, Carlos, Navas, Plácido

“…Coenzyme Q10 (CoQ10) deficiency has been associated with an increasing number of clinical phenotypes that respond to CoQ10 supplementation. In two siblings…”
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UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases by Suárez-Rivero, Juan M, Pastor-Maldonado, Carmen J, Povea-Cabello, Suleva, Ãlvarez-Córdoba, Mónica, Villalón-García, Irene, Talaverón-Rey, Marta, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Reche-López, Diana, Cilleros-Holgado, Paula, Piéero-Perez, Rocío, Sánchez-Alcázar, José A

“…Mitochondrial diseases represent one of the most common groups of genetic diseases. With a prevalence greater than 1 in 5000 adults, such diseases still lack…”
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Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels by Talaverón-Rey, Marta, Álvarez-Córdoba, Mónica, Villalón-García, Irene, Povea-Cabello, Suleva, Suárez-Rivero, Juan M, Gómez-Fernández, David, Romero-González, Ana, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Cilleros-Holgado, Paula, Reche-López, Diana, Piñero-Pérez, Rocío, Sánchez-Alcázar, José A

“…Neurodegeneration with brain iron accumulation (NBIA) disorders are a group of neurodegenerative diseases that have in common the accumulation of iron in the…”
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Antioxidants Prevent Iron Accumulation and Lipid Peroxidation, but Do Not Correct Autophagy Dysfunction or Mitochondrial Bioenergetics in Cellular Models of BPAN by Suárez-Carrillo, Alejandra, Álvarez-Córdoba, Mónica, Romero-González, Ana, Talaverón-Rey, Marta, Povea-Cabello, Suleva, Cilleros-Holgado, Paula, Piñero-Pérez, Rocío, Reche-López, Diana, Gómez-Fernández, David, Romero-Domínguez, José Manuel, Munuera-Cabeza, Manuel, Díaz, Antonio, González-Granero, Susana, García-Verdugo, José Manuel, Sánchez-Alcázar, José A

“…Neurodegeneration with brain iron accumulation (NBIA) is a group of rare neurogenetic disorders frequently associated with iron accumulation in the basal…”
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Pantothenate Rescues Iron Accumulation in Pantothenate Kinase-Associated Neurodegeneration Depending on the Type of Mutation by Álvarez-Córdoba, Mónica, Fernández Khoury, Aida, Villanueva-Paz, Marina, Gómez-Navarro, Carmen, Villalón-García, Irene, Suárez-Rivero, Juan M., Povea-Cabello, Suleva, de la Mata, Mario, Cotán, David, Talaverón-Rey, Marta, Pérez-Pulido, Antonio J., Salas, Joaquín J., Pérez-Villegas, Eva Mª, Díaz-Quintana, Antonio, Armengol, José A., Sánchez-Alcázar, José A.

“…Neurodegeneration with brain iron accumulation (NBIA) is a group of inherited neurologic disorders in which iron accumulates in the basal ganglia resulting in…”
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Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives by Álvarez-Córdoba, Mónica, Talaverón-Rey, Marta, Villalón-García, Irene, Povea-Cabello, Suleva, Suárez-Rivero, Juan M, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Salas, Joaquín J, Sánchez-Alcázar, José A

“…Neurodegeneration with brain iron accumulation (NBIA) is a group of genetic neurological disorders frequently associated with iron accumulation in the basal…”
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Coenzyme Q deficiency triggers mitochondria degradation by mitophagy by Rodríguez-Hernández, Ángeles, Cordero, Mario D., Salviati, Leonardo, Artuch, Rafael, Pineda, Mercé, Briones, Paz, Gómez Izquierdo, Lourdes, Cotán, David, Navas, Plácido, Sánchez-Alcázar, José A.

“…Coenzyme Q 10 (CoQ) is a small lipophilic molecule critical for the transport of electrons from complexes I and II to complex III in the mitochondrial…”
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From Mitochondria to Atherosclerosis: The Inflammation Path by Suárez-Rivero, Juan M, Pastor-Maldonado, Carmen J, Povea-Cabello, Suleva, Álvarez-Córdoba, Mónica, Villalón-García, Irene, Talaverón-Rey, Marta, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Sánchez-Alcázar, José A

“…Inflammation is a key process in metazoan organisms due to its relevance for innate defense against infections and tissue damage. However, inflammation is also…”
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Pterostilbene in Combination With Mitochondrial Cofactors Improve Mitochondrial Function in Cellular Models of Mitochondrial Diseases by Suárez-Rivero, Juan M, Pastor-Maldonado, Carmen J, Romero-González, Ana, Gómez-Fernandez, David, Povea-Cabello, Suleva, Álvarez-Córdoba, Mónica, Villalón-García, Irene, Talaverón-Rey, Marta, Suárez-Carrillo, Alejandra, Munuera-Cabeza, Manuel, Sánchez-Alcázar, José A

“…Mitochondrial diseases are genetic disorders caused by mutations in genes in the nuclear DNA (nDNA) and mitochondrial DNA (mtDNA) that encode mitochondrial…”
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AMPK Regulation of Cell Growth, Apoptosis, Autophagy, and Bioenergetics by Villanueva-Paz, Marina, Cotán, David, Garrido-Maraver, Juan, Oropesa-Ávila, Manuel, de la Mata, Mario, Delgado-Pavón, Ana, de Lavera, Isabel, Alcocer-Gómez, Elizabet, Álvarez-Córdoba, Mónica, Sánchez-Alcázar, José A

“…In eukaryotic cells, AMP-activated protein kinase (AMPK) generally promotes catabolic pathways that produce ATP and at the same time inhibits anabolic pathways…”
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Coenzyme Q10 Analogues: Benefits and Challenges for Therapeutics by Suárez-Rivero, Juan M., Pastor-Maldonado, Carmen J., Povea-Cabello, Suleva, Álvarez-Córdoba, Mónica, Villalón-García, Irene, Munuera-Cabeza, Manuel, Suárez-Carrillo, Alejandra, Talaverón-Rey, Marta, Sánchez-Alcázar, José A.

“…Coenzyme Q10 (CoQ10 or ubiquinone) is a mobile proton and electron carrier of the mitochondrial respiratory chain with antioxidant properties widely used as an…”
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