107395 20240104111815.0 doi 10.1111/bph.15276 sideral 120102 ART-2021-120102 eng Manzano, Raquel Universidad de Zaragoza (orcid)0000-0002-7477-8742 What skeletal muscle has to say in amyotrophic lateral sclerosis: implications for therapy 2021 Access copy available to the general public Unrestricted Amyotrophic lateral sclerosis (ALS) is an adult onset disorder characterised by progressive neuromuscular junction (NMJ) dismantling and degeneration of motor neurons leading to atrophy and paralysis of voluntary muscles responsible for motion and breathing. Except for a minority of patients harbouring genetic mutations, the origin of most ALS cases remains elusive. Peripheral tissues, and particularly skeletal muscle, have lately demonstrated an active contribution to disease pathology attracting a growing interest for these tissues as therapeutic targets in ALS. In this sense molecular mechanisms essential for cell and tissue homeostasis have been shown to be de‐regulated in the disease. These include muscle metabolism and mitochondrial activity, RNA processing, tissue‐resident stem cell function responsible for muscle regeneration, and proteostasis that regulates muscle mass in adulthood. This review aims to compile scientific evidence that demonstrates the role of skeletal muscle in ALS pathology and serve as reference for development of novel therapeutic strategies targeting this tissue to delay disease onset and progression. info:eu-repo/grantAgreement/ES/FIS-FEDER/PI17-00949 info:eu-repo/grantAgreement/EC/H2020/752349 /EU/Nanoparticle-based immunization, a novel therapeutic strategy for amyotrophic lateral sclerosis/NanoALS This project has received funding from the European Union’s Horizon 2020 research and innovation program under grant agreement No H2020 752349-NanoALS info:eu-repo/semantics/openAccess All rights reserved http://www.europeana.eu/rights/rr-f/ 9.473 2021 PHARMACOLOGY & PHARMACY 18 / 279 = 0.065 2021 Q1 T1 1.993 2021 Pharmacology 2021 Q1 13.6 2021 info:eu-repo/semantics/review info:eu-repo/semantics/acceptedVersion Toivonen, Janne Markus Universidad de Zaragoza (orcid)0000-0002-7243-1737 Moreno-Martínez, Laura Universidad de Zaragoza (orcid)0000-0002-7277-4318 de la Torre, Miriam Universidad de Zaragoza (orcid)0000-0002-7955-7164 Moreno-García, Leticia Universidad de Zaragoza (orcid)0000-0003-2436-7205 López-Royo,Tresa Universidad de Zaragoza (orcid)0000-0001-9802-8199 Molina, Nora Zaragoza, Pilar Universidad de Zaragoza (orcid)0000-0001-5740-0185 Calvo, Ana Cristina Universidad de Zaragoza (orcid)0000-0001-5193-7782 Osta, Rosario Universidad de Zaragoza (orcid)0000-0001-5687-6704 1001 420 Universidad de Zaragoza Dpto. Anatom.,Embri.Genét.Ani. Área Genética 1012 315 Universidad de Zaragoza Dpto. Farmac.Fisiol.y Med.L.F. Área Farmacología 178, 6 (2021), 1279-1297 Br. J. Pharmacol. BRITISH JOURNAL OF PHARMACOLOGY 0007-1188 1296837 http://zaguan.unizar.es/record/107395/files/texto_completo.pdf Postprint 1860461 http://zaguan.unizar.es/record/107395/files/texto_completo.jpg?subformat=icon icon Postprint oai:zaguan.unizar.es:107395 articulos driver 2024-01-04-11:04:36 ARTICLE