{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T17:01:08Z","timestamp":1789750868326,"version":"4.0.1"},"reference-count":67,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2021,7,8]],"date-time":"2021-07-08T00:00:00Z","timestamp":1625702400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100015496","name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas","doi-asserted-by":"publisher","award":["CB18\/05\/00040"],"award-info":[{"award-number":["CB18\/05\/00040"]}],"id":[{"id":"10.13039\/501100015496","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Health Research Fund of the Spanish Ministry of Economy and Competitiveness and the European Union (ERDF)","award":["PI16\/01325, PI17\/01841 and DTS19\/00061"],"award-info":[{"award-number":["PI16\/01325, PI17\/01841 and DTS19\/00061"]}]},{"name":"GENE (Association of Neuromuscular diseases of Gipuzkoa)","award":["Without number"],"award-info":[{"award-number":["Without number"]}]},{"name":"Department of Education, Universities and Research of the Basque Government","award":["PRE-2016-1-0382"],"award-info":[{"award-number":["PRE-2016-1-0382"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Limb-girdle muscular dystrophy R1 calpain 3-related (LGMDR1) is an autosomal recessive muscular dystrophy produced by mutations in the CAPN3 gene. It is a rare disease and there is no cure or treatment for the disease while the pathophysiological mechanism by which the absence of calpain 3 provokes the dystrophy in muscles is not clear. However, key proteins implicated in Wnt and mTOR signaling pathways, which regulate muscle homeostasis, showed a considerable reduction in their expression and in their phosphorylation in LGMDR1 patients\u2019 muscles. Finally, the administration of tideglusib and VP0.7, ATP non-competitive inhibitors of glycogen synthase kinase 3\u03b2 (GSK-3\u03b2), restore the expression and phosphorylation of these proteins in LGMDR1 cells, opening the possibility of their use as therapeutic options.<\/jats:p>","DOI":"10.3390\/ijms22147367","type":"journal-article","created":{"date-parts":[[2021,7,8]],"date-time":"2021-07-08T10:42:17Z","timestamp":1625740937000},"page":"7367","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Allosteric Modulation of GSK-3\u03b2 as a New Therapeutic Approach in Limb Girdle Muscular Dystrophy R1 Calpain 3-Related"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3002-6688","authenticated-orcid":false,"given":"Anabel","family":"Rico","sequence":"first","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garazi","family":"Guembelzu","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1473-4086","authenticated-orcid":false,"given":"Valle","family":"Palomo","sequence":"additional","affiliation":[{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"},{"name":"Centro de Investigaciones Biol\u00f3gicas Margarita Salas CSIC, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2707-8110","authenticated-orcid":false,"given":"Ana","family":"Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"},{"name":"Centro de Investigaciones Biol\u00f3gicas Margarita Salas CSIC, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Aiastui","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Cell Culture and Histology Platform, 20014 San Sebastian, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7504-6578","authenticated-orcid":false,"given":"Leire","family":"Casas-Fraile","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9211-4034","authenticated-orcid":false,"given":"Andrea","family":"Valls","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9509-4032","authenticated-orcid":false,"given":"Adolfo","family":"L\u00f3pez de Munain","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"},{"name":"Osakidetza Basque Health Service, Donostialdea Integrated Health Organisation, Department of Neurology, 20014 San Sebastian, Spain"},{"name":"Department of Neurosciences, University of the Basque Country UPV-EHU, 20014 San Sebastian, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0704-1150","authenticated-orcid":false,"given":"Amets","family":"S\u00e1enz","sequence":"additional","affiliation":[{"name":"Biodonostia Health Research Institute, Neurosciences Area, 20014 San Sebastian, Spain"},{"name":"Spanish Ministry of Economy & Competitiveness, Carlos III Health Institute, CIBERNED, 28031 Madrid, Spain"},{"name":"Osakidetza Basque Health Service, Donostialdea Integrated Health Organisation, Department of Neurology, 20014 San Sebastian, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,7,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1093\/brain\/119.1.295","article-title":"Juvenile limb-girdle muscular dystrophy. 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