000129648 001__ 129648
000129648 005__ 20241125101133.0
000129648 0247_ $$2doi$$a10.3390/ijms242115783
000129648 0248_ $$2sideral$$a135871
000129648 037__ $$aART-2023-135871
000129648 041__ $$aeng
000129648 100__ $$aGonzalo-Gobernado, Rafael
000129648 245__ $$aRepaglinide induces 003726 processing and neuroprotection in transgenic SOD1G93A mice
000129648 260__ $$c2023
000129648 5060_ $$aAccess copy available to the general public$$fUnrestricted
000129648 5203_ $$aThe 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.
000129648 536__ $$9info:eu-repo/grantAgreement/ES/ISCIII/FEDER/PI21-00372
000129648 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000129648 590__ $$a4.9$$b2023
000129648 592__ $$a1.179$$b2023
000129648 591__ $$aBIOCHEMISTRY & MOLECULAR BIOLOGY$$b66 / 313 = 0.211$$c2023$$dQ1$$eT1
000129648 593__ $$aMedicine (miscellaneous)$$c2023$$dQ1
000129648 591__ $$aCHEMISTRY, MULTIDISCIPLINARY$$b68 / 231 = 0.294$$c2023$$dQ2$$eT1
000129648 593__ $$aPhysical and Theoretical Chemistry$$c2023$$dQ1
000129648 593__ $$aComputer Science Applications$$c2023$$dQ1
000129648 593__ $$aInorganic Chemistry$$c2023$$dQ1
000129648 593__ $$aSpectroscopy$$c2023$$dQ1
000129648 593__ $$aOrganic Chemistry$$c2023$$dQ1
000129648 593__ $$aMolecular Biology$$c2023$$dQ2
000129648 593__ $$aCatalysis$$c2023$$dQ2
000129648 594__ $$a8.1$$b2023
000129648 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000129648 700__ $$0(orcid)0000-0002-7277-4318$$aMoreno-Martínez, Laura$$uUniversidad de Zaragoza
000129648 700__ $$aGonzález, Paz
000129648 700__ $$aDopazo, Xose Manuel
000129648 700__ $$0(orcid)0000-0001-5193-7782$$aCalvo, Ana Cristina$$uUniversidad de Zaragoza
000129648 700__ $$aPidal-Ladrón de Guevara, Isabel
000129648 700__ $$aSeisdedos, Elisa
000129648 700__ $$aDíaz-Muñoz, Rodrigo
000129648 700__ $$aMellström, Britt
000129648 700__ $$0(orcid)0000-0001-5687-6704$$aOsta, Rosario$$uUniversidad de Zaragoza
000129648 700__ $$aNaranjo, José Ramón
000129648 7102_ $$11001$$2420$$aUniversidad de Zaragoza$$bDpto. Anatom.,Embri.Genét.Ani.$$cÁrea Genética
000129648 7102_ $$11012$$2315$$aUniversidad de Zaragoza$$bDpto. Farmac.Fisiol.y Med.L.F.$$cÁrea Farmacología
000129648 773__ $$g24, 21 (2023), 15783 [16 pp.]$$pInt. j. mol. sci.$$tInternational Journal of Molecular Sciences$$x1661-6596
000129648 8564_ $$s3960470$$uhttps://zaguan.unizar.es/record/129648/files/texto_completo.pdf$$yVersión publicada
000129648 8564_ $$s2848281$$uhttps://zaguan.unizar.es/record/129648/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000129648 909CO $$ooai:zaguan.unizar.es:129648$$particulos$$pdriver
000129648 951__ $$a2024-11-22-11:59:34
000129648 980__ $$aARTICLE