Search Results - "Ledesma, Dolores"

Models to study basic and applied aspects of lysosomal storage disorders by Gaudioso, Ángel, Silva, Teresa P., Dolores Ledesma, María

“…© 2022 The Author(s). Published by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). The lack…”
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Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses by Baixauli, Francesc, Acín-Pérez, Rebeca, Villarroya-Beltrí, Carolina, Mazzeo, Carla, Nuñez-Andrade, Norman, Gabandé-Rodriguez, Enrique, Ledesma, Maria Dolores, Blázquez, Alberto, Martin, Miguel Angel, Falcón-Pérez, Juan Manuel, Redondo, Juan Miguel, Enríquez, Jose Antonio, Mittelbrunn, Maria

“…The endolysosomal system is critical for the maintenance of cellular homeostasis. However, how endolysosomal compartment is regulated by mitochondrial function…”
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Lipid changes in the aged brain: Effect on synaptic function and neuronal survival by Ledesma, María Dolores, Martin, Mauricio G., Dotti, Carlos G.

“…As the brain ages, cognitive and motor performance decline. This decline is thought to be largely due to the accumulation of damaging products from normal…”
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SGPL1 (sphingosine phosphate lyase 1) modulates neuronal autophagy via phosphatidylethanolamine production by Mitroi, Daniel N., Karunakaran, Indulekha, Gräler, Markus, Saba, Julie D., Ehninger, Dan, Ledesma, María Dolores, van Echten-Deckert, Gerhild

“…Macroautophagy/autophagy defects have been identified as critical factors underlying the pathogenesis of neurodegenerative diseases. The roles of the bioactive…”
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Brain cholesterol in normal and pathological aging by Martin, Mauricio, Dotti, Carlos G., Ledesma, Maria Dolores

“…Correct lipid homeostasis at the plasma membrane is essential for cell survival and performance. These are critically challenged in the aging brain. Changes in…”
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Lysosomal cell death mechanisms in aging by Gómez-Sintes, Raquel, Ledesma, María Dolores, Boya, Patricia

“…Highlights • Lysosomal cell death participates in physiological processes, aging, and disease. • Lysosomal membrane permeabilization plays a key role in…”
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Modulation of Dietary Choline Uptake in a Mouse Model of Acid Sphingomyelinase Deficiency by Gaudioso, Ángel, Moreno-Huguet, Pilar, Casas, Josefina, Schuchman, Edward H, Ledesma, María Dolores

“…Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder caused by mutations in the gene-encoding acid sphingomyelinase (ASM). ASMD impacts…”
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Sphingomyelin 16:0 is a therapeutic target for neuronal death in acid sphingomyelinase deficiency by Gaudioso, Ángel, Jiang, Xuntian, Casas, Josefina, Schuchman, Edward H., Ledesma, María Dolores

“…Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder caused by mutations in the SMPD1 gene encoding for the acid sphingomyelinase (ASM)…”
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Ellagic acid and its metabolites urolithins A/B ameliorate most common disease phenotypes in cellular and mouse models for lysosomal storage disorders by enhancing extracellular vesicle secretion by Soto-Huelin, Beatriz, Babiy, Bohdan, Pastor, Oscar, Díaz-García, Mario, Toledano-Zaragoza, Ana, Frutos, María Dolores, Espín, Juan Carlos, Tomás-Barberán, Francisco A., Busto, Rebeca, Ledesma, María Dolores

“…Niemann Pick diseases types A (NPDA) and C (NPDC) are lysosomal storage disorders (LSDs) leading to cognitive impairment, neurodegeneration, and early death…”
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Addressing neurodegeneration in lysosomal storage disorders: Advances in Niemann Pick diseases by Toledano-Zaragoza, Ana, Ledesma, María Dolores

“…Most lysosomal storage disorders (LSDs) cause progressive neurodegeneration leading to early death. While the genetic defects that cause these disorders impact…”
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Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice by Ledesma, Maria Dolores, Prinetti, Alessandro, Sonnino, Sandro, Schuchman, Edward H

“…J. Neurochem. (2011) 116, 779-788. ABSTRACT: Severe neurological involvement characterizes Niemann Pick disease (NPD) type A, an inherited disorder caused by…”
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Host sphingomyelin increases West Nile virus infection in vivo by Martín-Acebes, Miguel A., Gabandé-Rodríguez, Enrique, García-Cabrero, Ana M., Sánchez, Marina P., Ledesma, María Dolores, Sobrino, Francisco, Saiz, Juan-Carlos

“…Flaviviruses, such as the dengue virus and the West Nile virus (WNV), are arthropod-borne viruses that represent a global health problem. The flavivirus…”
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Enhanced mGluR5 intracellular activity causes psychiatric alterations in Niemann Pick type C disease by Toledano-Zaragoza, Ana, Enriquez-Zarralanga, Violeta, Naya-Forcano, Sara, Briz, Víctor, Alfaro-Ruíz, Rocío, Parra-Martínez, Miguel, Mitroi, Daniel N., Luján, Rafael, Esteban, José A., Ledesma, María Dolores

“…Niemann-Pick disease Type C (NPC) is caused by mutations in the cholesterol transport protein NPC1 leading to the endolysosomal accumulation of the lipid and…”
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Conventional and molecular marker-assisted selection and pyramiding of genes for multiple disease resistance in tomato by Hanson, Peter, Lu, Shu-Fen, Wang, Jaw-Fen, Chen, Wallace, Kenyon, Lawrence, Tan, Chee-Wee, Tee, Kwee Lian, Wang, Ya-Ying, Hsu, Yun-Che, Schafleitner, Roland, Ledesma, Dolores, Yang, Ray-Yu

“…•Five tomato lines resistant to six diseases were developed at AVRDC Taiwan.•Selection was done by molecular markers, seedling inoculations, and field…”
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Inhibition of fatty acid amide hydrolase prevents pathology in neurovisceral acid sphingomyelinase deficiency by rescuing defective endocannabinoid signaling by Bartoll, Adrián, Toledano‐Zaragoza, Ana, Casas, Josefina, Guzmán, Manuel, Schuchman, Edward H, Ledesma, María Dolores

“…Acid sphingomyelinase deficiency (ASMD) leads to cellular accumulation of sphingomyelin (SM), neurodegeneration, and early death. Here, we describe the…”
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Lipid dynamics at dendritic spines by Dotti, Carlos Gerardo, Esteban, Jose Antonio, Ledesma, María Dolores

“…Dynamic changes in the structure and composition of the membrane protrusions forming dendritic spines underlie memory and learning processes. In recent years a…”
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CERTL reduces C16 ceramide, amyloid-β levels, and inflammation in a model of Alzheimer’s disease by Crivelli, Simone M., Luo, Qian, Stevens, Jo A.A., Giovagnoni, Caterina, van Kruining, Daan, Bode, Gerard, den Hoedt, Sandra, Hobo, Barbara, Scheithauer, Anna-Lena, Walter, Jochen, Mulder, Monique T., Exley, Christopher, Mold, Matthew, Mielke, Michelle M., De Vries, Helga E., Wouters, Kristiaan, van den Hove, Daniel L. A., Berkes, Dusan, Ledesma, María Dolores, Verhaagen, Joost, Losen, Mario, Bieberich, Erhard, Martinez-Martinez, Pilar

“…Abstract Background Dysregulation of ceramide and sphingomyelin levels have been suggested to contribute to the pathogenesis of Alzheimer’s disease (AD)…”
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Improvement of biochemical and behavioral defects in the Niemann–Pick type A mouse by intraventricular infusion of MARCKS by Trovò, Laura, Stroobants, Stijn, D'Hooge, Rudi, Ledesma, Maria Dolores, Dotti, Carlos G

“…Abstract Niemann–Pick disease type A (NPDA) is a fatal disease due to mutations in the acid sphingomyelinase (ASM) gene, which triggers the abnormal…”
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NPC1 enables cholesterol mobilization during long‐term potentiation that can be restored in Niemann–Pick disease type C by CYP46A1 activation by Mitroi, Daniel N, Pereyra‐Gómez, Guadalupe, Soto‐Huelin, Beatriz, Senovilla, Fernando, Kobayashi, Toshihide, Esteban, Jose A, Ledesma, María Dolores

“…NPC is a neurodegenerative disorder characterized by cholesterol accumulation in endolysosomal compartments. It is caused by mutations in the gene encoding…”
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Fruit Quality and Bioactive Compounds with Antioxidant Activity of Tomatoes Grown On-Farm: Comparison of Organic and Conventional Management Systems by Juroszek, Peter, Lumpkin, Heidi M, Yang, Ray-Yu, Ledesma, Dolores R, Ma, Chin-Hua

“…Replicated field trials at three matched farm pairs in southern and central Taiwan were established in October 2004 and 2005 to compare fruit quality and…”
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