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Your search keyword '"Vila, Miquel"' showing total 324 results
Start Over Author "Vila, Miquel" Publication Type Academic Journals
324 results on '"Vila, Miquel"'

1. Modelling human neuronal catecholaminergic pigmentation in rodents recapitulates age-related neurodegenerative deficits

Author

Laguna, Ariadna, Peñuelas, Núria, Gonzalez-Sepulveda, Marta, Nicolau, Alba, Arthaud, Sébastien, Guillard-Sirieix, Camille, Lorente-Picón, Marina, Compte, Joan, Miquel-Rio, Lluís, Xicoy, Helena, Liu, Jiong, Parent, Annabelle, Cuadros, Thais, Romero-Giménez, Jordi, Pujol, Gemma, Giménez-Llort, Lydia, Fort, Patrice, Bortolozzi, Analia, Carballo-Carbajal, Iria, and Vila, Miquel

Published
2024
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6. Lysosomal lipid alterations caused by glucocerebrosidase deficiency promote lysosomal dysfunction, chaperone-mediated-autophagy deficiency, and alpha-synuclein pathology

Author

Navarro-Romero, Alba, Fernandez-Gonzalez, Irene, Riera, Jordi, Montpeyo, Marta, Albert-Bayo, Merce, Lopez-Royo, Tresa, Castillo-Sanchez, Pablo, Carnicer-Caceres, Clara, Arranz-Amo, Jose Antonio, Castillo-Ribelles, Laura, Pradas, Eddie, Casas, Josefina, Vila, Miquel, and Martinez-Vicente, Marta

Published
2022
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9. Basic Science in Movement Disorders: Fueling the Engine of Translation into Clinical Practice.

Author

Outeiro, Tiago F., Kalia, Lorraine V., Bezard, Erwan, Ferrario, Juan, Lin, Chin‐Hsien, Salama, Mohamed, Standaert, David G., Taiwo, Lolade, Takahashi, Ryosuke, Vila, Miquel, Mollenhauer, Brit, and Svenningsson, Per

Abstract

Basic Science is crucial for the advancement of clinical care for Movement Disorders. Here, we provide brief updates on how basic science is important for understanding disease mechanisms, disease prevention, disease diagnosis, development of novel therapies and to establish the basis for personalized medicine. We conclude the viewpoint by a call to action to further improve interactions between clinician and basic scientists. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. [ABSTRACT FROM AUTHOR]

Published
2024
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17. Resistance of α-Synuclein Null Mice to the Parkinsonian Neurotoxin MPTP

Author

Dauer, William, Kholodilov, Nikolai, Vila, Miquel, Trillat, Anne-Cecile, Goodchild, Rose, Larsen, Kristin E., Staal, Roland, Tieu, Kim, Schmitz, Yvonne, Yuan, Chao Annie, Rocha, Marcelo, Jackson-Lewis, Vernice, Hersch, Steven, Sulzer, David, Przedborski, Serge, Burke, Robert, and Hen, Rene

Published
2002

18. Sex-dependent calcium hyperactivity due to lysosomal-related dysfunction in astrocytes from APOE4 versus APOE3 gene targeted replacement mice

Author

Larramona-Arcas, Raquel, González-Arias, Candela, Perea, Gertrudis, Gutiérrez, Antonia, Vitorica, Javier, García-Barrera, Tamara, Gómez-Ariza, José Luis, Pascua-Maestro, Raquel, Ganfornina, María Dolores, Kara, Eleanna, Hudry, Eloise, Martinez-Vicente, Marta, Vila, Miquel, Galea, Elena, and Masgrau, Roser

Published
2020
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21. Neuromelanin accumulation drives endogenous synucleinopathy in non-human primates.

Author

Chocarro, Julia, Rico, Alberto J, Ariznabarreta, Goiaz, Roda, Elvira, Honrubia, Adriana, Collantes, María, Peñuelas, Iván, Vázquez, Alfonso, Rodríguez-Pérez, Ana I, Labandeira-García, José L, Vila, Miquel, and Lanciego, José L

Subjects
DOPAMINERGIC neurons, PRIMATES, SUBSTANTIA nigra, PARKINSON'S disease, GENETIC vectors
Abstract

Although neuromelanin is a dark pigment characteristic of dopaminergic neurons in the human substantia nigra pars compacta, its potential role in the pathogenesis of Parkinson's disease (PD) has often been neglected since most commonly used laboratory animals lack neuromelanin. Here we took advantage of adeno-associated viral vectors encoding the human tyrosinase gene for triggering a time-dependent neuromelanin accumulation within substantia nigra pars compacta dopaminergic neurons in macaques up to similar levels of pigmentation as observed in elderly humans. Furthermore, neuromelanin accumulation induced an endogenous synucleinopathy mimicking intracellular inclusions typically observed in PD together with a progressive degeneration of neuromelanin-expressing dopaminergic neurons. Moreover, Lewy body-like intracellular inclusions were observed in cortical areas of the frontal lobe receiving dopaminergic innervation, supporting a circuit-specific anterograde spread of endogenous synucleinopathy by permissive trans-synaptic templating. In summary, the conducted strategy resulted in the development and characterization of a new macaque model of PD matching the known neuropathology of this disorder with unprecedented accuracy. Most importantly, evidence is provided showing that intracellular aggregation of endogenous α-synuclein is triggered by neuromelanin accumulation, therefore any therapeutic approach intended to decrease neuromelanin levels may provide appealing choices for the successful implementation of novel PD therapeutics. [ABSTRACT FROM AUTHOR]

Published
2023
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22. The Small GTPase RAC1/CED-10 Is Essential in Maintaining Dopaminergic Neuron Function and Survival Against α-Synuclein-Induced Toxicity

Author

Kim, Hanna, Calatayud, Carles, Guha, Sanjib, Fernández-Carasa, Irene, Berkowitz, Laura, Carballo-Carbajal, Iria, Ezquerra, Mario, Fernández-Santiago, Rubén, Kapahi, Pankaj, Raya, Ángel, Miranda-Vizuete, Antonio, Lizcano, Jose Miguel, Vila, Miquel, Caldwell, Kim A., Caldwell, Guy A., Consiglio, Antonella, and Dalfo, Esther

Published
2018
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27. Aberrant epigenome in iPSC‐derived dopaminergic neurons from Parkinson's disease patients

Author

Fernández‐Santiago, Rubén, Carballo‐Carbajal, Iria, Castellano, Giancarlo, Torrent, Roger, Richaud, Yvonne, Sánchez‐Danés, Adriana, Vilarrasa‐Blasi, Roser, Sánchez‐Pla, Alex, Mosquera, José Luis, Soriano, Jordi, López‐Barneo, José, Canals, Josep M, Alberch, Jordi, Raya, Ángel, Vila, Miquel, Consiglio, Antonella, Martín‐Subero, José I, Ezquerra, Mario, and Tolosa, Eduardo

Published
2015
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30. Adult-specific Reelin expression alters striatal neuronal organization: implications for neuropsychiatric disorders.

Author

Pardo, Mònica, Gregorio, Sara, Montalban, Enrica, Pujadas, Lluís, Elias-Tersa, Alba, Masachs, Núria, Vílchez-Acosta, Alba, Parent, Annabelle, Auladell, Carme, Girault, Jean-Antoine, Vila, Miquel, Nairn, Angus C., Manso, Yasmina, and Soriano, Eduardo

Subjects
NEUROBEHAVIORAL disorders, INTERNEURONS, EXTRACELLULAR matrix proteins, OPIOID receptors, AUTISM spectrum disorders, MENTAL illness, TOURETTE syndrome
Abstract

In addition to neuronal migration, brain development, and adult plasticity, the extracellular matrix protein Reelin has been extensively implicated in human psychiatric disorders such as schizophrenia, bipolar disorder, and autism spectrum disorder. Moreover, heterozygous reeler mice exhibit features reminiscent of these disorders, while overexpression of Reelin protects against its manifestation. However, how Reelin influences the structure and circuits of the striatal complex, a key region for the above-mentioned disorders, is far from being understood, especially when altered Reelin expression levels are found at adult stages. In the present study, we took advantage of complementary conditional gain- and loss-of-function mouse models to investigate how Reelin levels may modify adult brain striatal structure and neuronal composition. Using immunohistochemical techniques, we determined that Reelin does not seem to influence the striatal patch and matrix organization (studied by m-opioid receptor immunohistochemistry) nor the density of medium spiny neurons (MSNs, studied with DARPP-32). We show that overexpression of Reelin leads to increased numbers of striatal parvalbumin- and cholinergic-interneurons, and to a slight increase in tyrosine hydroxylase-positive projections. We conclude that increased Reelin levels might modulate the numbers of striatal interneurons and the density of the nigrostriatal dopaminergic projections, suggesting that these changes may be involved in the protection of Reelin against neuropsychiatric disorders. [ABSTRACT FROM AUTHOR]

Published
2023
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31. In vivo reduction of age-dependent neuromelanin accumulation mitigates features of Parkinson's disease.

Author

Gonzalez-Sepulveda, Marta, Compte, Joan, Cuadros, Thais, Nicolau, Alba, Guillard-Sirieix, Camille, Peñuelas, Núria, Lorente-Picon, Marina, Parent, Annabelle, Romero-Giménez, Jordi, Cladera-Sastre, Joana M, Laguna, Ariadna, and Vila, Miquel

Subjects
PARKINSON'S disease, MONOAMINE transporters, SUBSTANTIA nigra, DOPAMINERGIC neurons, PATHOLOGY, GENETIC vectors
Abstract

Humans accumulate with age the dark-brown pigment neuromelanin inside specific neuronal groups. Neurons with the highest neuromelanin levels are particularly susceptible to degeneration in Parkinson's disease, especially dopaminergic neurons of the substantia nigra, the loss of which leads to characteristic motor Parkinson's disease symptoms. In contrast to humans, neuromelanin does not appear spontaneously in most animals, including rodents, and Parkinson's disease is an exclusively human condition. Using humanized neuromelanin-producing rodents, we recently found that neuromelanin can trigger Parkinson's disease pathology when accumulated above a specific pathogenic threshold. Here, by taking advantage of this newly developed animal model, we assessed whether the intracellular build-up of neuromelanin that occurs with age can be slowed down in vivo to prevent or attenuate Parkinson's disease. Because neuromelanin derives from the oxidation of free cytosolic dopamine, we enhanced dopamine vesicular encapsulation in the substantia nigra of neuromelanin-producing rats by viral vector-mediated overexpression of vesicular monoamine transporter 2 (VMAT2). This strategy reduced the formation of potentially toxic oxidized dopamine species that can convert into neuromelanin and maintained intracellular neuromelanin levels below their pathogenic threshold. Decreased neuromelanin production was associated with an attenuation of Lewy body-like inclusion formation and a long-term preservation of dopamine homeostasis, nigrostriatal neuronal integrity and motor function in these animals. Our results demonstrate the feasibility and therapeutic potential of modulating age-dependent intracellular neuromelanin production in vivo , thereby opening an unexplored path for the treatment of Parkinson's disease and, in a broader sense, brain ageing. [ABSTRACT FROM AUTHOR]

Published
2023
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34. Correction to: The Small GTPase RAC1/CED-10 Is Essential in Maintaining Dopaminergic Neuron Function and Survival Against α-Synuclein-Induced Toxicity

Author

Kim, Hanna, Calatayud, Carles, Guha, Sanjib, Fernández-Carasa, Irene, Berkowitz, Laura, Carballo-Carbajal, Iria, Ezquerra, Mario, Fernández-Santiago, Rubén, Kapahi, Pankaj, Raya, Ángel, Miranda-Vizuete, Antonio, Lizcano, Jose Miguel, Vila, Miquel, Caldwell, Kim A., Caldwell, Guy A., Consiglio, Antonella, and Dalfo, Esther

Published
2018
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40. Disease‐specific phenotypes in dopamine neurons from human iPS‐based models of genetic and sporadic Parkinson's disease

Author

Sánchez‐Danés, Adriana, Richaud‐Patin, Yvonne, Carballo‐Carbajal, Iria, Jiménez‐Delgado, Senda, Caig, Carles, Mora, Sergio, Di Guglielmo, Claudia, Ezquerra, Mario, Patel, Bindiben, Giralt, Albert, Canals, Josep M., Memo, Maurizio, Alberch, Jordi, López‐Barneo, José, Vila, Miquel, Cuervo, Ana Maria, Tolosa, Eduard, Consiglio, Antonella, and Raya, Angel

Published
2012
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44. Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage

Author

Perier, Celine, Tieu, Kim, Guegan, Christelle, Caspersen, Casper, Jackson-Lewis, Vernice, Carelli, Valerio, Martinuzzi, Andrea, Hirano, Michio, Przedborski, Serge, and Vila, Miquel

Subjects
Mitochondria -- Research, Parkinson's disease -- Research, Nervous system -- Degeneration, Nervous system -- Research, Science and technology
Abstract

Dysfunction of mitochondrial complex I is a feature of human neurodegenerative diseases such as Leber hereditary optic neuropathy and Parkinson's disease. This mitochondrial defect is associated with a recruitment of the mitochondrial-dependent apoptotic pathway in vivo. However, in isolated brain mitochondria, complex I dysfunction caused by either pharmacological or genetic means fails to directly activate this cell death pathway. Instead, deficits of complex I stimulate intramitochondrial oxidative stress, which, in turn, increase the releasable soluble pool of cytochrome c within the mitochondrial intermembrane space. Upon mitochondrial permeabilization by the cell death agonist Bax, more cytochrome c is released to the cytosol from brain mitochondria with impaired complex I activity. Given these results, we propose a model in which defects of complex I lower the threshold for activation of mitochondrial-dependent apoptosis by Bax, thereby rendering compromised neurons more prone to degenerate. This molecular scenario may have far-reaching implications for the development of effective neuroprotective therapies for these incurable illnesses. mitochondria | neurodegeneration | Parkinson's disease

Published
2005

50. Brain injections of glial cytoplasmic inclusions induce a multiple system atrophy-like pathology.

Author

Teil, Margaux, Dovero, Sandra, Bourdenx, Mathieu, Arotcarena, Marie-Laure, Camus, Sandrine, Porras, Gregory, Thiolat, Marie-Laure, Trigo-Damas, Ines, Perier, Celine, Estrada, Cristina, Garcia-Carrillo, Nuria, Morari, Michele, Meissner, Wassilios G, Herrero, María Trinidad, Vila, Miquel, Obeso, Jose A, Bezard, Erwan, and Dehay, Benjamin

Subjects
CELL metabolism, BRAIN, LEWY body dementia, NERVE tissue proteins, DEMYELINATION, ANIMAL experimentation, MEDIUM spiny neurons, PARKINSON'S disease, RESEARCH funding, NEURODEGENERATION
Abstract

Synucleinopathies encompass several neurodegenerative diseases, which include Parkinson's disease, dementia with Lewy bodies and multiple system atrophy. These diseases are characterized by the deposit of α-synuclein aggregates in intracellular inclusions in neurons and glial cells. Unlike Parkinson's disease and dementia with Lewy bodies, where aggregates are predominantly neuronal, multiple system atrophy is associated with α-synuclein cytoplasmic inclusions in oligodendrocytes. Glial cytoplasmic inclusions are the pathological hallmark of multiple system atrophy and are associated with neuroinflammation, modest demyelination and, ultimately, neurodegeneration. To evaluate the possible pathogenic role of glial cytoplasmic inclusions, we inoculated glial cytoplasmic inclusion-containing brain fractions obtained from multiple system atrophy patients into the striatum of non-human primates. After a 2-year in vivo phase, extensive histochemical and biochemical analyses were performed on the whole brain. We found loss of both nigral dopamine neurons and striatal medium spiny neurons, as well as loss of oligodendrocytes in the same regions, which are characteristics of multiple system atrophy. Furthermore, demyelination, neuroinflammation and α-synuclein pathology were also observed. These results show that the α-synuclein species in multiple system atrophy-derived glial cytoplasmic inclusions can induce a pathological process in non-human primates, including nigrostriatal and striatofugal neurodegeneration, oligodendroglial cell loss, synucleinopathy and gliosis. The present data pave the way for using this experimental model for MSA research and therapeutic development. [ABSTRACT FROM AUTHOR]

Published
2022
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