X-ALD): Control of Redox/Metabolic Homeostasis and Inflammation

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X-ALD): Control of Redox/Metabolic Homeostasis and Inflammation NRF2 and RIP140 as new therapeutic targets for X-linked adrenoleukodystrophy (X-ALD): Control of redox/metabolic homeostasis and inflammation Pablo Ranea Robles Aquesta tesi doctoral està subjecta a la llicència Reconeixement 3.0. Espanya de Creative Commons. Esta tesis doctoral está sujeta a la licencia Reconocimiento 3.0. España de Creative Commons. This doctoral thesis is licensed under the Creative Commons Attribution 3.0. Spain License. AGRADECIMIENTOS (ACKNOWLEDGMENTS) Aviso a lectores. Tengo mucha gente a la que agradecer, así que reservad un buen rato para poder leer y cotillear todos los agradecimientos. Quiero agradecer en primer lugar, cómo no, a mis dos directores de tesis, Aurora y Stéphane. Aunque hayamos tenido nuestros más y nuestros menos, como los hay en cualquier relación profesional, esta tesis no hubiera sido posible sin la ayuda de vosotros. A Aurora quiero agradecerle la oportunidad que me dio, cuando recién salido de la licenciatura de Biología en Granada confió en mí para empezar esta aventura. A lo largo de estos años he aprendido, a base de “palos”, en el buen sentido de la palabra, a ser minucioso en los detalles, a ser escéptico de mis propios resultados, a no darme por vencido, a sacarme las castañas del fuego por mí mismo y sobre todo, a creer en mí mismo a la hora de presentar mi trabajo a los demás. Eso, en parte, se lo debo a Aurora, y quiero agradecérselo. Sin embargo, también es verdad que sin Stéphane esta tesis nunca hubiera visto la luz. Por temas logísticos y estancias de Aurora en el extranjero, al final la hemos sacado codo a codo los dos. Gracias por aguantar mi infinidad de correos, por estar pendiente y por dejar expresar libremente mi opinión y tenerla en cuenta a la hora de seguir con los proyectos. Sinceramente, creo que los dos hemos aprendido uno del otro, después de tantas horas de trabajo y de correcciones. Y no me olvido a Isidro, por tutorizar esta tesis como miembro UB. Después quiero agradecer a todas las agencias financiadoras que han hecho posible esta tesis, desde la primera beca lanzadera del CIBERER hasta la beca PFIS del Instituto de Salud Carlos III. Ojalá de aquí unos años el resto de contratos (¡ya no se dice becas!) se pudieran siquiera equiparar a las PFIS, que comparadas a FPU, FPIS y becas autonómicas está muy por encima. Aunque tal y cómo está el país, muchas cosas tendrían que cambiar, desgraciadamente. Puede parecer que la tesis se queda en nosotros tres, pero todos sabemos que el trabajo sale adelante en este laboratorio gracias al inmenso equipo humano que forma X-ALD. Sinceramente, no sé cómo hubiera sobrevivido a lo duro que es este trabajo sin todos vosotros, que me habéis hecho sentir como en familia. Desde las más seniors en el lab, como Montse, que ha vivido todo mi cambio personal desde aquel chavalillo que llegó desde Granada y que me ha enseñado múltiples técnicas enzimáticas como una auténtica maestra; o Agatha, de la que aún me sigue flipando todo lo que sabe. Un auténtico diamante que la mayoría no conoce pero sin ella muchos proyectos del lab nunca hubieran surgido. Y qué decir de Nath, una de las mejores personas que he podido conocer (y es que por este laboratorio han pasado algunas de ellas) y de la que estoy orgulloso de poder llevarme su amistad una vez acabe mi etapa aquí. El día en que todo ese trabajo y ese buen trato hacia las personas sean reconocidos justamente está muy cercano, estoy muy seguro. También quiero agradecer a los otros postdocs, como los Xavi (Joya y Ortega), doktor Pohl, el recién llegado Edgard, que siempre han estado ahí para hacer más ameno el día a día en el lab y, muchas veces, fuera de él. Por supuesto gracias a el equipo técnico. Muchas veces no éramos conscientes de todo lo que sacan adelante, aunque últimamente un poco más y creo que aquí puedo hablar en primera persona, desde que se fueron Bárbara y Jordi. Es un gustazo poder trabajar con gente como Laia, Cris y Juanjo. Ya les gustaría a la mayoría de predocs del mundo poder tener a gente así en sus labs. La amabilidad de Cris y su disponibilidad a colaborar siempre hacen el día a día más sencillo y agradable. Sobre Laia, creo que puedo decir v con seguridad que pocas veces veré a nadie más eficiente, rápida y todoterreno como Laia “Chicho Terremoto”, aunque ahora sus prestaciones hayan bajado como es normal con la pequeña Nura, a la que espero poder ver en la defensa de esta tesis :) Un capítulo especial merece Juanjo, no hay duda. Junto con mis compis de piso, la primera persona que me “acogió” aquí en Barcelona, me enseñó infinidad de sitios, siempre dispuesto a ayudar, a ir a cualquier sitio. He aprendido de fotografía, de coches, bueno de todo, ¡porque Juanjo sabe de todo! Una persona especial para mí, y que echaré de menos seguro de aquí a unos meses. ¿Con quién voy a discutir ahora sobre cualquier tema random? No me olvido tampoco de mis compañeros y compañeras de faena, “The Indian Crew”, la famiglia y la nueva generación. Quien iba a imaginar que acabaría hablando inglés tanto tiempo aquí en Barcelona con mis tres compañeros indios, Janani, Devesh y Sanjib. Thanks people for always being nice to me, and for showing me another culture, for those stories about your cities, the weddings, the traditions, that I always liked. I hope that our paths would meet again somewhere else, eating a berlina in whatever cafetería we may meet. With Janani I spent more years than with the others, and we are finishing at the same time more or less. It has been a great pleasure to share these years with you, Janani. You are unique, extremely brave and I’m sure that you’ll get what you want, just keep fighting for it. Kundi power! ¿Qué voy a decir de la famiglia? Me faltan las palabras, junto con mis compis de piso, son mi otra familia aquí en Barcelona. Gracias, Patri y Andrea, por tantos buenos ratos, tanto dentro como fuera del laboratorio, por esos viajes con Juanjo y Kelly (que no me olvido tampoco) que nunca olvidaré. Aún tengo pendiente una visita a Catanzaro y a la Puglia a conocer a Beatrice y a saludar a mi colega Lorenzo, que estoy seguro también me echa de menos. Y por supuesto a Ale y Marco, con Ale compartí mis vivencias en Barcelona desde el principio, ya que llegamos casi a la vez, y he podido disfrutar de momentos maravillosos como su boda en Italia con Andrea, y los primeros meses del pequeño Lore. También incluyo aquí en la famiglia a Sonia. Otra persona muy especial, a la que hay que conocer y que cuando lo haces descubres a una persona maravillosa y a una buena amiga. Venga, más visitas pendientes, se me llena la agenda en un plis, Valencia y Bogotá (centro), todo a su tiempo. Y la nueva generación, encabezada por Leire. Ya has demostrado que vales para esto, así que nada, para adelante como haces ahora, que 4 años se pasan volando y hay que aprovechar y disfrutar de todos los momentos. Seguro que ahora van llegando compañeros y compañeras que harán más llevadero todo este viaje. Si son gente como Laura, o como Sergi, seguro tendrás una experiencia tan buena como la mía ;) Ay, mis maestros y maestras cuando llegué al lab: Jone, Jorge y Laia, ahora todos en el extranjero. Fue una suerte teneros cerca cuando llegué para adaptarme, entender muchas cosas de las que pasaban a mi alrededor y aprender casi todo lo que ahora sé después de 5 años de tesis. Aunque nuestros caminos se hayan separado, siempre pueden volver a cruzarse, fijaos si habrá dado de sí esta tesis que le ha dado tiempo a Jorge a irse a Inglaterra y volver y todo. Siempre lo digo, pero otra de las personas que me han dejado huella ha sido Jone, otro torrente de “buen rollo” con la que hablar de cualquier cosa y de la que se puede aprender y debatir sobre miles de cosas. Estoy seguro que en San Diego están súper contentos de tenerte allí, y espero que volvamos a coincidir pronto. vi Ahora que he nombrado Jorge, y ya saliéndome del lab, tengo que mencionar a mis hermanos/as aquí en Barcelona. Mis compañeros de piso Laura y Fran. Llegué por casualidad al piso a través de Jorge cuando visité el laboratorio, y acabo llevándome dos buenísimos amigos, sin los cuales todos estos años en Barcelona no hubieran sido iguales. Una amistad que hace que incluso Fran me perdone la sucesora que le dejé en el piso. Gracias a toda la gente de Genética y del IDIBELL. Una de las cosas que me llevo de mi experiencia aquí, es poder haber estado “en mi salsa”, en un ambiente cercano, en el que la mayoría nos conocíamos, aunque fuéramos muchos y en distintos departamentos. Con gente como Antonia o Javi (tenemos que mantener esa guerra del Supermanager estemos dónde estemos), o Mercè, Virginia, Sara, Julián, Xavi, Montse Porta, Ana, Ina, Gema...; compañeros y compañeras como Xènia, Carmen, Clara, Javi y muchos y muchas más que me olvidaré pero que tengo en cuenta, es que si no estos agradecimientos van a ocupar media tesis. Gente que ya pasó por GM pero que dejó huella, como Eva, otra persona maravillosa (os dije que había varias que habían pasado por aquí) a la que tengo la suerte de poder seguir viendo, gente como Esther, Sergio, Itziar, Álvaro, Eric, Curro o Iris.
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