Por favor, use este identificador para citar o enlazar a este item:
http://hdl.handle.net/10261/338350
COMPARTIR / EXPORTAR:
SHARE CORE BASE | |
Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL | DATACITE | |
Título: | Repaglinide Induces ATF6 Processing and Neuroprotection in Transgenic SOD1G93A Mice |
Autor: | Gonzalo-Gobernado, Rafael CSIC ORCID; Moreno-Martínez, Laura; González Pérez, Paz; Dopazo, Xose M.; Calvo, Ana Cristina; Pidal-Ladrón de Guevara, Isabel; Seisdedos, Elisa; Díaz-Muñoz, Rodrigo; Mellström, Britt CSIC; Osta, Rosario; Naranjo, José Ramón CSIC ORCID | Palabras clave: | ALS Repaglinide DREAM ATF6 SOD1 UPR Motoneurons Microglia Astroglia |
Fecha de publicación: | 1-nov-2023 | Editor: | Multidisciplinary Digital Publishing Institute | Citación: | International Journal of Molecular Sciences 24(21): 15783 (2023) | Resumen: | The interaction of the activating transcription factor 6 (ATF6), a key effector of the unfolded protein response (UPR) in the endoplasmic reticulum, with the neuronal calcium sensor Downstream Regulatory Element Antagonist Modulator (DREAM) is a potential therapeutic target in neurodegeneration. Modulation of the ATF6–DREAM interaction with repaglinide (RP) induced neuroprotection in a model of Huntington’s disease. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with no cure, characterized by the progressive loss of motoneurons resulting in muscle denervation, atrophy, paralysis, and death. The aim of this work was to investigate the potential therapeutic significance of DREAM as a target for intervention in ALS. We found that the expression of the DREAM protein was reduced in the spinal cord of SOD1G93A mice compared to wild-type littermates. RP treatment improved motor strength and reduced the expression of the ALS progression marker collagen type XIXα1 (Col19α1 mRNA) in the quadriceps muscle in SOD1G93A mice. Moreover, treated SOD1G93A mice showed reduced motoneuron loss and glial activation and increased ATF6 processing in the spinal cord. These results indicate that the modulation of the DREAM–ATF6 interaction ameliorates ALS symptoms in SOD1G93A mice. | Versión del editor: | https://doi.org/10.3390/ijms242115783 | URI: | http://hdl.handle.net/10261/338350 | DOI: | 10.3390/ijms242115783 | ISSN: | 1661-6596 | E-ISSN: | 1422-0067 |
Aparece en las colecciones: | (CNB) Artículos |
Ficheros en este ítem:
Fichero | Descripción | Tamaño | Formato | |
---|---|---|---|---|
ijms-24-15783-v2.pdf | 3,87 MB | Adobe PDF | Visualizar/Abrir |
CORE Recommender
Page view(s)
31
checked on 28-abr-2024
Download(s)
21
checked on 28-abr-2024
Google ScholarTM
Check
Altmetric
Altmetric
Este item está licenciado bajo una Licencia Creative Commons