Search results for "EBE"
showing 10 items of 929 documents
Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery
2020
Spinocerebellar ataxia type-1 (SCA1) is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are somehow linked to neuronal death. However, owing to lack of a suitable cellular model, the downstream consequences of IIB formation are yet to be resolved. Here, we describe a nuclear protein aggregation model of pathogenic human ataxin-1 and characterize IIB effects. Using an inducible Sleeping Beauty transposon system, we overexpressed the ATXN1(Q82) gene in human mesenchymal stem cells that are resistant to the early cytotoxic effects caused by the expr…
Circadian Rhythm Variations and Nutrition in Children
2018
AbstractCircadian rhythms are the changes in biological processes that occur on a daily basis. Among these processes are reactions involved in metabolic homeostasis. Circadian rhythms are structured by the central clock in the suprachiasmatic nucleus of the hypothalamus via the control of melatonin expression. Circadian rhythms are also controlled by the peripheral clocks, which are intracellular mechanisms composed of the clock genes, whose expression follows a circadian pattern. Circadian rhythms are impacted by signals from the environment called zeitgebers, or time givers, which include light exposure, feeding schedule and composition, sleeping schedule and pattern, temperature, and phy…
MECP2 impairs neuronal structure by regulating KIBRA
2016
Using a Drosophila model of MECP2 gain-of-function, we identified memory associated KIBRA as a target of MECP2 in regulating dendritic growth. We found that expression of human MECP2 increased kibra expression in Drosophila, and targeted RNAi knockdown of kibra in identified neurons fully rescued dendritic defects as induced by MECP2 gain-of-function. Validation in mouse confirmed that Kibra is similarly regulated by Mecp2 in a mammalian system. We found that Mecp2 gain-of-function in cultured mouse cortical neurons caused dendritic impairments and increased Kibra levels. Accordingly, Mecp2 loss-of-function in vivo led to decreased Kibra levels in hippocampus, cortex, and cerebellum. Togeth…
Dynamics of a Protein Interaction Network Associated to the Aggregation of polyQ-Expanded Ataxin-1
2020
Background: Several experimental models of polyglutamine (polyQ) diseases have been previously developed that are useful for studying disease progression in the primarily affected central nervous system. However, there is a missing link between cellular and animal models that would indicate the molecular defects occurring in neurons and are responsible for the disease phenotype in vivo. Methods: Here, we used a computational approach to identify dysregulated pathways shared by an in vitro and an in vivo model of ATXN1(Q82) protein aggregation, the mutant protein that causes the neurodegenerative polyQ disease spinocerebellar ataxia type-1 (SCA1). Results: A set of common dysregulated pathwa…
Pedaleo de brazos en personas con lesión medular, parálisis cerebral o ataxia cerebelosa: Parámetros fisiológicos. [Armcrank pedaling in persons with…
2016
espanolLos desordenes neurologicos generan afectacion fisica y derivan en sedentarismo, enfermedades coronarias y obesidad o diabetes, reduciendo tanto la esperanza como la calidad de vida. La oferta de actividad fisica adaptada es escasa por falta de informacion especifica sobre la forma de adecuarlo y dosificarlo a las personas que presentan estos desordenes. Con el fin de comparar el efecto del ejercicio de pedaleo de brazos sobre la respuesta fisiologica y la percepcion del esfuerzo, en 8 personas con lesion medular, 4 con paralisis cerebral y 4 con ataxia de Friedreich, se analizo su respuesta fisiologica, asi como su percepcion subjetiva al esfuerzo, frente a un grupo Control (16 part…
Kildare rebellion (1534-1535) in the Annals of the Four Masters
2020
Abstract One of the most important Irish historical sources, which are the Annals of the Four Masters, written in the modern period, provide us with unusually valuable information about the history of the Emerald Island. In addition to data from the ancient or medieval periods, it also contains material from the difficult 16th and 17th centuries for Ireland, when it came under the yoke of English Protestants, who were initially represented by the Tudors and then by the Stuart dynasty. The Annals of the Four Masters also witnesses the resistance of the Irish, both those from Hiberno Normans and Gaelic Irish, to the demands of new authorities, including the rejection of Catholicism and total …
Musical training predicts cerebello-hippocampal coupling during music listening.
2018
Cerebello-hippocampal interactions occur during accurate spatiotemporal prediction of movements. In the context of music listening, differences in cerebello-hippocampal functional connectivity may result from differences in predictive listening accuracy. Using functional MRI, we studied differences in this network between 18 musicians and 18 nonmusicians while they listened to music. Musicians possess a predictive listening advantage over nonmusicians, facilitated by strengthened coupling between produced and heard sounds through lifelong musical experience. Thus, we hypothesized that musicians would exhibit greater functional connectivity than nonmusicians as a marker of accurate online pr…
La temporalidad de la pensión compensatoria en la jurisprudencia del tribunal supremo
2021
En el presente trabajo se estudian los criterios jurisprudenciales en orden a determinar si una pensión compensatoria debe tener carácter indefinido o temporal.
Children’s freedom in the family: childrens’ duties and parents’ right of correction. Situation in Spanish Law
2016
En este trabajo se analiza la regulación de los deberes de los hijos y el derecho de corrección de los padres tras las últimas reformas legislativas, para determinar si sigue existiendo ese ius corrigendi pese a su eliminación formal del Código civil español, aunque no de otras legislaciones forales. In this paper we study the regulation of children’s duties and the parents’ right of correction after the last legislative reforms, to determinate if continues existing this ius corrigendi despite the formal elimination from the Spanish civil code, though not of other statutory legislations.
Reflexiones a propósito de la protección de datos en el escenario global digital: El derecho de daños en la litigiosidad internacional
2021
La circulación internacional de datos personales constituye un sector pujante en la esfera socio-económica. En tales condiciones, ante la existencia de un tratamiento ilícito de aquellos, el agraviado está legitimado para exigir una indemnización por los daños y perjuicios que se hubieran podido originar en el marco transfronterizo. Tal situación plantea dos cuestiones fundamentales: por un lado, determinar el órgano jurisdiccional competente para conocer de un eventual litigio, y, por otro, precisar la Ley aplicable para resolver la controversia suscitada. En la presente investigación cabe destacar las significativas novedades que introduce el Reglamento (UE) 2016/679, de 27 de abril de 20…