{"id":284723,"date":"2024-08-29T08:02:40","date_gmt":"2024-08-29T06:02:40","guid":{"rendered":"https:\/\/dhcp205002.cbm.uam.es\/index.php\/programas-cientificos\/procesos-fisiologicos-y-patologicos\/neuropatologia-molecular\/fisiopatologia-y-terapia-de-las-enfermedades-neurodegenerativas-ataxia-de-friedreich\/"},"modified":"2024-08-29T08:21:09","modified_gmt":"2024-08-29T06:21:09","slug":"fisiopatologia-y-terapia-de-las-enfermedades-neurodegenerativas-ataxia-de-friedreich","status":"publish","type":"page","link":"https:\/\/www.cbm.uam.es\/index.php\/programas-cientificos\/procesos-fisiologicos-y-patologicos\/neuropatologia-molecular\/fisiopatologia-y-terapia-de-las-enfermedades-neurodegenerativas-ataxia-de-friedreich\/?lang=es","title":{"rendered":"Fisiopatolog\u00eda y Terapia de las Enfermedades Neurodegenerativas: Ataxia de Friedreich"},"content":{"rendered":"<p>[et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;#34609B&#8221; background_image=&#8221;https:\/\/www.cbm.uam.es\/wp-content\/uploads\/2024\/07\/iStock-1356994685small-scaled.jpg&#8221; background_position=&#8221;bottom_center&#8221; background_vertical_offset=&#8221;32%&#8221; min_height=&#8221;352px&#8221; height=&#8221;294px&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row column_structure=&#8221;2_5,3_5&#8243; use_custom_gutter=&#8221;on&#8221; gutter_width=&#8221;4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;RGBA(255,255,255,0)&#8221; custom_margin=&#8221;61px||||false|false&#8221; custom_padding=&#8221;||0px|||&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;2_5&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;rgba(52,96,155,0.83)&#8221; custom_padding=&#8221;50px||50px|50px|false|false&#8221; border_radii=&#8221;on|15px|15px|15px|15px&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; text_text_color=&#8221;#FFFFFF&#8221; custom_margin=&#8221;0px||10px||false|false&#8221; custom_padding=&#8221;0px||0px||false|false&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p>Programa Cient\u00edfico<\/p>\n<p>[\/et_pb_text][et_pb_heading title=&#8221;Procesos fisiol\u00f3gicos y patol\u00f3gicos&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; title_font=&#8221;Montserrat||||||||&#8221; title_text_color=&#8221;#FFFFFF&#8221; title_font_size=&#8221;43px&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][\/et_pb_column][et_pb_column type=&#8221;3_5&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; custom_padding=&#8221;157px||||false|false&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h4 style=\"text-align: center;\"><span style=\"color: #13b5bd;\">UNIDADES EN ESTE PROGRAMA <\/span><\/h4>\n<hr>\n<h6 style=\"text-align: center;\"><span style=\"color: #13b5bd;\"><a href=\"https:\/\/www.cbm.uam.es\/index.php\/programas-cientificos\/procesos-fisiologicos-y-patologicos\/neuropatologia-molecular\/?lang=es\">Neuropatolog\u00eda molecular<\/a><\/span>           <span style=\"color: #abb8c3;\"><a href=\"https:\/\/www.cbm.uam.es\/index.php\/programas-cientificos\/procesos-fisiologicos-y-patologicos\/redes-metabolicas-y-senalizadoras-en-la-enfermedad\/?lang=es\">Redes metab\u00f3licas y de se\u00f1alizaci\u00f3n en la enfermedad<\/a><\/span><\/h6>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; 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global_colors_info=&#8221;{}&#8221;][et_pb_heading title=&#8221;GRUPO DE INVESTIGACI\u00d3N&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; title_level=&#8221;h5&#8243; title_text_color=&#8221;#13B5BD&#8221; custom_margin=&#8221;50px||0px||false|false&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][et_pb_blurb title=&#8221;Fisiopatolog\u00eda y Terapia de las Enfermedades Neurodegenerativas: Ataxia de Friedreich&#8221; use_icon=&#8221;on&#8221; font_icon=&#8221;&#xe046;||divi||400&#8243; icon_color=&#8221;#13B5BD&#8221; icon_placement=&#8221;left&#8221; image_icon_width=&#8221;26px&#8221; content_max_width=&#8221;1100px&#8221; content_max_width_tablet=&#8221;1100px&#8221; content_max_width_last_edited=&#8221;off|desktop&#8221; _builder_version=&#8221;4.27.0&#8243; header_level=&#8221;h1&#8243; header_font=&#8221;Montserrat||||||||&#8221; header_text_color=&#8221;#000000&#8243; header_font_size=&#8221;29px&#8221; header_line_height=&#8221;1.4em&#8221; body_font=&#8221;||||||||&#8221; body_font_size=&#8221;15px&#8221; body_line_height=&#8221;1.8em&#8221; image_icon_custom_margin=&#8221;6px||||false|false&#8221; background_layout=&#8221;dark&#8221; custom_margin=&#8221;|||&#8221; custom_padding=&#8221;||14px||false|false&#8221; animation_style=&#8221;slide&#8221; animation_direction=&#8221;right&#8221; animation_intensity_slide=&#8221;10%&#8221; animation=&#8221;off&#8221; image_max_width=&#8221;64px&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_blurb][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;rgba(175,218,249,0.18)&#8221; custom_padding=&#8221;0px||0px||false|false&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row column_structure=&#8221;1_4,3_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;\/imgs\/default.jpg&#8221; title_text=&#8221;logoRecurso 7&#8243; align=&#8221;right&#8221; module_class=&#8221;roundedImg&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][et_pb_column type=&#8221;3_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_heading title=&#8221;Javier Diaz-Nido&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; title_level=&#8221;h3&#8243; title_text_align=&#8221;left&#8221; title_text_color=&#8221;#008C93&#8243; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;-27px||||false|false&#8221; link_option_url_new_window=&#8221;on&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p style=\"text-align: left;\">\n<a href=\"https:\/\/orcid.org\/0000-0002-0927-7925\" target=\"_blank\" rel=\"noopener\">ORCID<\/a><\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;#13B5BD&#8221; custom_margin=&#8221;0px||0px||false|false&#8221; custom_padding=&#8221;0px||0px||false|false&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row column_structure=&#8221;1_3,1_3,1_3&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;1_3&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_button button_url=&#8221;#investiga&#8221; button_text=&#8221;Investigaci\u00f3n&#8221; button_alignment=&#8221;center&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_button=&#8221;on&#8221; button_bg_color=&#8221;#34609B&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_button][\/et_pb_column][et_pb_column type=&#8221;1_3&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_button button_url=&#8221;#personal&#8221; button_text=&#8221;Miembros del grupo&#8221; button_alignment=&#8221;center&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_button=&#8221;on&#8221; button_bg_color=&#8221;#34609B&#8221; custom_padding=&#8221;|44px||44px|false|true&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_button][\/et_pb_column][et_pb_column type=&#8221;1_3&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_button button_url=&#8221;#publica&#8221; button_text=&#8221;Publicaciones&#8221; button_alignment=&#8221;center&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_button=&#8221;on&#8221; button_bg_color=&#8221;#34609B&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_button][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_enable_image=&#8221;off&#8221; background_size=&#8221;custom&#8221; background_image_height=&#8221;84%&#8221; background_position=&#8221;bottom_right&#8221; custom_margin=&#8221;0px||0px||false|false&#8221; custom_padding=&#8221;0px||15px||false|false&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row column_structure=&#8221;1_2,1_2&#8243; use_custom_gutter=&#8221;on&#8221; gutter_width=&#8221;2&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;20px||||false|false&#8221; custom_padding=&#8221;||||false|false&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;1_2&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text admin_label=&#8221;resumen&#8221; module_id=&#8221;resumen&#8221; module_class=&#8221;resumen&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; text_font_size=&#8221;15px&#8221; text_line_height=&#8221;1.8em&#8221; background_color=&#8221;#f1f8fe&#8221; custom_padding=&#8221;20px|20px|20px|20px|true|false&#8221; border_width_top=&#8221;1px&#8221; border_color_top=&#8221;#008C93&#8243; border_width_bottom=&#8221;1px&#8221; border_color_bottom=&#8221;#008C93&#8243; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p style=\"text-align: justify;\">Nuestro grupo se centra en el estudio de un tipo de enfermedades neurodegenerativas, las ataxias, que se caracterizan por la p\u00e9rdida de neuronas en el cerebelo y la m\u00e9dula espinal. En particular, nos hemos enfocado en la ataxia de Friedreich, que es la ataxia hereditaria m\u00e1s frecuente en Europa. Intentamos esclarecer las bases moleculares de esta patolog\u00eda y desarrollar terapias que puedan ser eficaces para su tratamiento.  <\/p>\n<p>[\/et_pb_text][\/et_pb_column][et_pb_column type=&#8221;1_2&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; custom_padding=&#8221;||||false|false&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;https:\/\/www.cbm.uam.es\/wp-content\/uploads\/2024\/08\/Image-207-Javier-Diaz-Nido.jpg&#8221; title_text=&#8221; &#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][\/et_pb_row][et_pb_row column_structure=&#8221;3_5,2_5&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;||||false|false&#8221; custom_padding=&#8221;0px||||false|false&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;3_5&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_heading title=&#8221;Investigaci\u00f3n&#8221; module_id=&#8221;investiga&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;0px|||0px|false|false&#8221; custom_padding=&#8221;3px|||8px|false|false&#8221; border_width_left=&#8221;6px&#8221; border_color_left=&#8221;#34609B&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p style=\"text-align: justify;\">La ataxia de Friedreich es una enfermedad neurogen\u00e9tica, autos\u00f3mica recesiva, causada por un d\u00e9ficit de frataxina, una prote\u00edna localizada mayoritariamente en las mitocondrias. Adem\u00e1s del componente neurodegenerativo, muchos pacientes padecen tambi\u00e9n de trastornos musculoesquel\u00e9ticos, cardiomiopat\u00eda hipertr\u00f3fica y diabetes. La ataxia de Friedreich es una enfermedad degenerativa de un inicio muy precoz, y puede servir como un modelo muy \u00fatil para otras enfermedades degenerativas en las que la disfunci\u00f3n mitocondrial tambi\u00e9n juega un papel muy importante.  <\/p>\n<p>[\/et_pb_text][\/et_pb_column][et_pb_column type=&#8221;2_5&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;data:image\/svg+xml;base64,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&#8221; title_text=&#8221; &#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;60px||||false|false&#8221; custom_padding=&#8221;|||27px||&#8221; animation_style=&#8221;zoom&#8221; border_width_all=&#8221;3px&#8221; border_color_all=&#8221;#13B5BD&#8221; box_shadow_style=&#8221;preset3&#8243; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; custom_padding=&#8221;0px||||false|false&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p style=\"text-align: justify;\">En el contexto de la ataxia de Friedreich, hemos desarrollado distintos modelos celulares neurales para estudiar los mecanismos moleculares del proceso neurodegenerativo disparado por la deficiencia de frataxina. Estos estudios nos han permitido identificar procesos como el d\u00e9ficit de centros hierro-azufre, la disminuci\u00f3n de algunos complejos de la cadena respiratoria mitocondrial, la generaci\u00f3n de especies reactivas de ox\u00edgeno, el incremento del da\u00f1o en el DNA, la activaci\u00f3n de la prote\u00edna p53, y la activaci\u00f3n de la apoptosis como elementos cruciales en la neurodegeneraci\u00f3n. Adem\u00e1s, hemos demostrado un papel adicional muy importante de la activaci\u00f3n de los astrocitos que contribuye al proceso neurodegenerativo a trav\u00e9s de procesos neuroinflamatorios y neurot\u00f3xicos.  <\/p>\n<p>[\/et_pb_text][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<p style=\"text-align: justify;\">Por otra parte, tambi\u00e9n estamos empleando nuestros modelos celulares para ensayar posibles estrategias terap\u00e9uticas con un \u00e9nfasis en encontrar mol\u00e9culas (f\u00e1rmacos o genes) capaces de compensar los d\u00e9ficits funcionales inducidos por la deficiencia de frataxina o bien capaces de incrementar la expresi\u00f3n de la frataxina de una manera eficiente. En particular estamos prestando una atenci\u00f3n especial a los factores neurotr\u00f3ficos y a los f\u00e1rmacos capaces de estimular la producci\u00f3n y\/o se\u00f1alizaci\u00f3n de dichos factores. Nuestro grup0 ha sido el primero en descubrir que el factor neurotr\u00f3fico derivado de cerebro (BDNF) tiene la capacidad de inhibir la neurodegeneraci\u00f3n inducida por la deficiencia de frataxina. Tambi\u00e9n hemos encontrado que SAG, un agonista de la v\u00eda de SHH, disminuye la activaci\u00f3n de los astrocitos deficientes de frataxina y la secreci\u00f3n de citoquinas proinflamatorias.<br \/>Nuestro grupo tambi\u00e9n ha sido pionero en el desarrollo de estrategias de terapia g\u00e9nica para la ataxia de Friedreich introduciendo copias correctas del gen de la frataxina mediante la administraci\u00f3n de vectores herpesvirales portadores del cDNA o del \u201clocus\u201d gen\u00f3mico completo de la frataxina.     <br \/>En la actualidad nuestro grupo est\u00e1 enfocado en la caracterizaci\u00f3n de la neurodegeneraci\u00f3n en el cerebelo de un nuevo modelo murino de ataxia de Friedreich. Este modelo murino tambi\u00e9n va a resultar muy \u00fatil para el test de posibles estrategias terap\u00e9uticas.<br \/>En colaboraci\u00f3n con el grupo del Dr. Tiago Fernandes y del Dr. Joaquim Cabral, del Instituto de Bioingenier\u00eda de la Universidade de Lisboa, tambi\u00e9n estamos explorando la generaci\u00f3n de organoides de cerebelo humano como un nuevo modelo experimental de la ataxia de Friedreich. Para ello estamos utilizando c\u00e9lulas iPS derivadas de biopsias de fibroblastos de sujetos sanos y pacientes con ataxia de Friedreich.<br \/>En el \u00e1mbito de la transferencia nuestro grupo ha tenido contratos de investigaci\u00f3n con la empresa biofarmac\u00e9utica Minoryx evaluando posibles f\u00e1rmacos en nuestros modelos de ataxia de Friedreich.<br \/>Javier Diaz-Nido desarrolla su actividad docente fundamentalmente en el Grado en Bioqu\u00edmica y en el Master en Biomedicina Molecular de la Universidad Aut\u00f3noma de Madrid. Tambi\u00e9n es miembro del Instituto de Derechos Humanos, Democracia, Cultura de Paz y No Violencia (DEMOS-PAZ) de la misma universidad. <\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;rgba(0,0,0,0.04)&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_heading title=&#8221;Miembros del grupo&#8221; module_id=&#8221;personal&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; custom_margin=&#8221;0px|||0px|false|false&#8221; custom_padding=&#8221;3px|||8px|false|false&#8221; border_width_left=&#8221;6px&#8221; border_color_left=&#8221;#34609B&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; max_width=&#8221;2560px&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_code _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_row _builder_version=\"4.25.0\" _module_preset=\"default\" column_structure=\"1_4,1_4,1_4,1_4\" hover_enabled=\"0\" sticky_enabled=\"0\"][et_pb_column _builder_version=\"4.25.0\" _module_preset=\"default\" type=\"1_4\"][et_pb_blurb title=\"Javier D\u00edaz Nido\" image=\"\/imgs\/default.jpg\" _builder_version=\"4.25.0\" _module_preset=\"default\" header_text_align=\"center\" body_text_align=\"center\" global_colors_info=\"{}\" theme_builder_area=\"post_content\" module_class=\"roundedImg\"]<p>Lab.: 207 Ext.: 4562<br \/>javier.diaznido(at)cbm.csic.es<\/p>[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][\/et_pb_code][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; 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border_width_left=&#8221;6px&#8221; border_color_left=&#8221;#34609B&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_heading][\/et_pb_column][\/et_pb_row][et_pb_row column_structure=&#8221;1_4,1_4,1_4,1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;https:\/\/www.cbm.uam.es\/wp-content\/uploads\/2024\/08\/JDN-Image1-Javier-Diaz-Nido.jpg&#8221; title_text=&#8221; &#8221; url_new_window=&#8221;on&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; link_option_url=&#8221;https:\/\/jneuroinflammation.biomedcentral.com\/articles\/10.1186\/s12974-022-02442-w&#8221; link_option_url_new_window=&#8221;on&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>The smoothened agonist SAG reduces mitochondrial dysfunction and neurotoxicity of frataxin-deficient astrocytes<\/h3>\n<p>Andr\u00e9s Vicente-Acosta et al.<\/p>\n<p>[\/et_pb_text][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;data:image\/svg+xml;base64,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&#8221; url_new_window=&#8221;on&#8221; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; link_option_url_new_window=&#8221;on&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>T\u00edtulo<\/h3>\n<p>Autores<\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row column_structure=&#8221;1_4,1_4,1_4,1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; animation_style=&#8221;zoom&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;https:\/\/www.cbm.uam.es\/wp-content\/uploads\/2024\/08\/JDN-Image-3-Javier-Diaz-Nido.jpg&#8221; title_text=&#8221; &#8221; url_new_window=&#8221;on&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; link_option_url=&#8221;https:\/\/www.cell.com\/molecular-therapy-family\/molecular-therapy\/fulltext\/S1525-0016(16)30215-5?&#8221; link_option_url_new_window=&#8221;on&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>Gene Transfer of Brain-derived Neurotrophic Factor (BDNF) Prevents Neurodegeneration Triggered by FXN Deficiency<\/h3>\n<p>Yurika Katsu-Jim\u00e9nez et al.<\/p>\n<p>[\/et_pb_text][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_image src=&#8221;https:\/\/www.cbm.uam.es\/wp-content\/uploads\/2024\/08\/JDN-Image-4-Javier-Diaz-Nido-scaled.jpg&#8221; title_text=&#8221; &#8221; url_new_window=&#8221;on&#8221; _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][\/et_pb_column][et_pb_column type=&#8221;1_4&#8243; _builder_version=&#8221;4.25.0&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.0&#8243; _module_preset=&#8221;default&#8221; link_option_url=&#8221;https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0888754315300021?&#8221; link_option_url_new_window=&#8221;on&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>Delivery of the 135 kb human frataxin genomic DNA locus gives rise to different frataxin isoforms<\/h3>\n<p>S. P\u00e9rez-Luz et al.<\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; background_color=&#8221;rgba(0,0,0,0.04)&#8221; da_disable_devices=&#8221;off|off|off&#8221; global_colors_info=&#8221;{}&#8221; da_is_popup=&#8221;off&#8221; da_exit_intent=&#8221;off&#8221; da_has_close=&#8221;on&#8221; da_alt_close=&#8221;off&#8221; da_dark_close=&#8221;off&#8221; da_not_modal=&#8221;on&#8221; da_is_singular=&#8221;off&#8221; da_with_loader=&#8221;off&#8221; da_has_shadow=&#8221;on&#8221;][et_pb_row _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.23.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_heading title=&#8221;\u00daltimas publicaciones&#8221; module_id=&#8221;publica&#8221; 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