000152987 001__ 152987
000152987 005__ 20251017144555.0
000152987 0247_ $$2doi$$a10.1016/j.nbd.2020.104793
000152987 0248_ $$2sideral$$a116489
000152987 037__ $$aART-2020-116489
000152987 041__ $$aeng
000152987 100__ $$aMòdol-Caballero, G.
000152987 245__ $$aGene therapy for overexpressing Neuregulin 1 type I in skeletal muscles promotes functional improvement in the SOD1G93A ALS mice
000152987 260__ $$c2020
000152987 5060_ $$aAccess copy available to the general public$$fUnrestricted
000152987 5203_ $$aAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting motoneurons (MNs), with no effective treatment currently available. The molecular mechanisms that are involved in MN death are complex and not fully understood, with partial contributions of surrounding glial cells and skeletal muscle to the disease. Neuregulin 1 (NRG1) is a trophic factor highly expressed in MNs and neuromuscular junctions. Recent studies have suggested a crucial role of the isoform I (NRG1-I) in the collateral reinnervation process in skeletal muscle, and NRG1-III in the preservation of MNs in the spinal cord, opening a window for developing novel therapies for neuromuscular diseases like ALS. In this study, we overexpressed NRG1-I widely in the skeletal muscles of the SOD1G93A transgenic mouse. The results show that NRG1 gene therapy activated the survival pathways in muscle and spinal cord, increasing the number of surviving MNs and neuromuscular junctions and reducing the astroglial reactivity in the spinal cord of the treated SOD1G93A mice. Furthermore, NRG1-I overexpression preserved motor function and delayed the onset of clinical disease. In summary, our data indicates that NRG1 plays an important role on MN survival and muscle innervation in ALS, and that viral-mediated overexpression of NRG1 isoforms may be considered as a promising approach for ALS treatment.
000152987 536__ $$9info:eu-repo/grantAgreement/ES/ISCIII-FEDER/PS09-720$$9info:eu-repo/grantAgreement/ES/ISCIII/MINECO/RD16-0011-0014
000152987 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttps://creativecommons.org/licenses/by/4.0/deed.es
000152987 590__ $$a5.996$$b2020
000152987 591__ $$aNEUROSCIENCES$$b54 / 273 = 0.198$$c2020$$dQ1$$eT1
000152987 592__ $$a2.205$$b2020
000152987 593__ $$aNeurology$$c2020$$dQ1
000152987 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000152987 700__ $$aHerrando-Grabulosa, M.
000152987 700__ $$aGarcía-Lareu, B.
000152987 700__ $$aSolanes, N.
000152987 700__ $$aVerdés, S.
000152987 700__ $$0(orcid)0000-0001-5687-6704$$aOsta, R.$$uUniversidad de Zaragoza
000152987 700__ $$aFrancos-Quijorna, I.
000152987 700__ $$aLópez-Vales, R.
000152987 700__ $$0(orcid)0000-0001-5193-7782$$aCalvo, A. C.$$uUniversidad de Zaragoza
000152987 700__ $$aBosch, A.
000152987 700__ $$aNavarro, X.
000152987 7102_ $$11001$$2420$$aUniversidad de Zaragoza$$bDpto. Anatom.,Embri.Genét.Ani.$$cÁrea Genética
000152987 773__ $$g137 (2020), 104793 [10 pp.]$$pNeurobiol. dis.$$tNeurobiology of disease$$x0969-9961
000152987 85641 $$uhttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85078985788&doi=10.1016%2fj.nbd.2020.104793&partnerID=40&md5=9d4978fbbbd57b99f1ed62a58a01219e$$zTexto completo de la revista
000152987 8564_ $$s2623399$$uhttps://zaguan.unizar.es/record/152987/files/texto_completo.pdf$$yVersión publicada
000152987 8564_ $$s2688521$$uhttps://zaguan.unizar.es/record/152987/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
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000152987 951__ $$a2025-10-17-14:12:48
000152987 980__ $$aARTICLE