{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T23:17:21Z","timestamp":1776554241019,"version":"3.51.2"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,1,27]],"date-time":"2022-01-27T00:00:00Z","timestamp":1643241600000},"content-version":"vor","delay-in-days":26,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Protocols"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Muscular dystrophies are caused by genetic variants in genes encoding for proteins important for muscle structure or function, leading to a loss of muscle integrity and muscle wasting. To this day, no cure has been found for these diseases. Different therapeutic approaches are under intensive investigation. Cellular therapy has been extensively studied for diseases such as Duchenne Muscular Dystrophy, a debilitating disease caused by a mutation in the <jats:italic>DMD<\/jats:italic> gene, encoding for the dystrophin protein. Healthy myogenic cells transplanted into dystrophic muscles have the potential to engraft at long\u2010term and fuse to donate their nuclei to the dystrophin\u2010deficient myofibers, thereby restoring normal gene expression. Despite promising preclinical studies, the clinical trials had limited success so far due to many technical limitations. The recent technological advances in induced\u2010pluripotent stem cells and genome editing opened new opportunities in this field. One of the keys to efficiently translate these new technologies into clinical benefits is to use relevant endpoints for preclinical studies. Considering that dystrophic muscles are susceptible to contraction\u2010induced injury, the assessment of their resistance to repeated eccentric contractions is an optimal outcome to evaluate their functional recovery following cell transplantation. This protocol describes the procedure to generate induced\u2010pluripotent stem cell\u2010derived myoblasts, transplant these cells into skeletal muscle of immunosuppressed dystrophic mice, and assess muscle function <jats:italic>in situ<\/jats:italic> by measuring the resistance of the transplanted muscle to repeated eccentric contractions. \u00a9 2022 Wiley Periodicals LLC.<\/jats:p><jats:p>This article was corrected on 4 August 2022. See the end of the full text for details.<\/jats:p><jats:p><jats:bold>Basic Protocol 1<\/jats:bold>: Generation of hiPSC\u2010derived myoblasts.<\/jats:p><jats:p><jats:bold>Basic Protocol 2<\/jats:bold>: Transplantation of hiPSC\u2010derived myoblasts in skeletal muscle of dystrophic mice.<\/jats:p><jats:p><jats:bold>Basic Protocol 3<\/jats:bold>: Assessment of muscle function <jats:italic>in situ<\/jats:italic><\/jats:p>","DOI":"10.1002\/cpz1.356","type":"journal-article","created":{"date-parts":[[2022,1,27]],"date-time":"2022-01-27T20:14:55Z","timestamp":1643314495000},"source":"Crossref","is-referenced-by-count":5,"title":["Assessment of Muscle Function Following hiPSC\u2010Derived Myoblast Transplantation in Dystrophic Mice"],"prefix":"10.1002","volume":"2","author":[{"given":"Paul","family":"Fabre","sequence":"first","affiliation":[{"name":"CHU Sainte\u2010Justine Research Center  Montreal Quebec Canada"},{"name":"Department of pharmacology and physiology, Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Molina","sequence":"additional","affiliation":[{"name":"CHU Sainte\u2010Justine Research Center  Montreal Quebec Canada"},{"name":"Department of pharmacology and physiology, Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zakaria","family":"Orfi","sequence":"additional","affiliation":[{"name":"CHU Sainte\u2010Justine Research Center  Montreal Quebec Canada"},{"name":"Department of pharmacology and physiology, Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicolas A.","family":"Dumont","sequence":"additional","affiliation":[{"name":"CHU Sainte\u2010Justine Research Center  Montreal Quebec Canada"},{"name":"School of rehabilitation, Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,1,27]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/stem.1402"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201310035"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.00803.2009"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2005.05.010"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/cphy.c140068"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.114223"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.115.6.1285"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1191035"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0147198"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41556\u2010017\u20100010\u20102"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.mt.6300295"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880171210"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00122.2021"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1155\/2017\/1376151"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpregu.1999.277.3.R917"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0046698"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13395\u2010020\u20100221\u20102"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/febs.14831"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/978\u20101\u201062703\u2010348\u20100_3"},{"issue":"1","key":"e_1_2_8_21_1","first-page":"CD003725","article-title":"Glucocorticoid corticosteroids for Duchenne muscular dystrophy","author":"Manzur A. 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