{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T16:12:31Z","timestamp":1787933551360,"version":"build-2784847793"},"reference-count":55,"publisher":"The Company of Biologists","issue":"20","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,10,15]]},"abstract":"<jats:p>Mice deficient in \u03b1-sarcoglycan (Sgca-null mice) develop progressive muscular dystrophy and serve as a model for human limb girdle muscular dystrophy type 2D. Sgca-null mice suffer a more severe myopathy than that of mdx mice, the model for Duchenne muscular dystrophy. This is the opposite of what is observed in humans and the reason for this is unknown. In an attempt to understand the cellular basis of this severe muscular dystrophy, we isolated clonal populations of myogenic progenitor cells (MPCs), the resident postnatal muscle progenitors of dystrophic and wild-type mice. MPCs from Sgca-null mice generated much smaller clones than MPCs from wild-type or mdx dystrophic mice. Impaired proliferation of Sgca-null myogenic precursors was confirmed by single fiber analysis and this difference correlated with Sgca expression during MPC proliferation. In the absence of dystrophin and associated proteins, which are only expressed after differentiation, SGCA complexes with and stabilizes FGFR1. Deficiency of Sgca leads to an absence of FGFR1 expression at the membrane and impaired MPC proliferation in response to bFGF. The low proliferation rate of Sgca-null MPCs was rescued by transduction with Sgca-expressing lentiviral vectors. When transplanted into dystrophic muscle, Sgca-null MPCs exhibited reduced engraftment. The reduced proliferative ability of Sgca-null MPCs explains, at least in part, the severity of this muscular dystrophy and also why wild-type donor progenitor cells engraft efficiently and consequently ameliorate disease.<\/jats:p>","DOI":"10.1242\/dev.070706","type":"journal-article","created":{"date-parts":[[2011,9,9]],"date-time":"2011-09-09T11:42:27Z","timestamp":1315568547000},"page":"4523-4533","source":"Crossref","is-referenced-by-count":27,"title":["Alpha sarcoglycan is required for FGF-dependent myogenic progenitor cell proliferation in vitro and in vivo"],"prefix":"10.1242","volume":"138","author":[{"given":"Marco","family":"Cassano","sequence":"first","affiliation":[{"name":"Laboratory of Translational Cardiomyology, Stem Cell Interdepartmental Institute, KU Leuven, Herestraat 49 O&N1 bus 814, 3000 Leuven, Belgium."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Dellavalle","sequence":"additional","affiliation":[{"name":"Division of Regenerative Medicine, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco Saverio","family":"Tedesco","sequence":"additional","affiliation":[{"name":"Division of Regenerative Medicine, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattia","family":"Quattrocelli","sequence":"additional","affiliation":[{"name":"Laboratory of Translational Cardiomyology, Stem Cell Interdepartmental Institute, KU Leuven, Herestraat 49 O&N1 bus 814, 3000 Leuven, Belgium."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Crippa","sequence":"additional","affiliation":[{"name":"Laboratory of Translational Cardiomyology, Stem Cell Interdepartmental Institute, KU Leuven, Herestraat 49 O&N1 bus 814, 3000 Leuven, Belgium."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flavio","family":"Ronzoni","sequence":"additional","affiliation":[{"name":"Human Anatomy, University of Pavia, Via Forlanini 8, 27100 Pavia, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnese","family":"Salvade","sequence":"additional","affiliation":[{"name":"Laboratory of Cell Therapy \u2018Stefano Verri\u2019 University of Milan-Bicocca, 20052 Monza, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Berardi","sequence":"additional","affiliation":[{"name":"Laboratory of Translational Cardiomyology, Stem Cell Interdepartmental Institute, KU Leuven, Herestraat 49 O&N1 bus 814, 3000 Leuven, Belgium."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvan","family":"Torrente","sequence":"additional","affiliation":[{"name":"Stem Cell Laboratory, Department of Neurological Science, Fondazione IRCCS C\u00e0 Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, University of Milan, 20122 Milan, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulio","family":"Cossu","sequence":"additional","affiliation":[{"name":"Division of Regenerative Medicine, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy."},{"name":"Department of Biology, University of Milan, Via Celoria 28, 20129 Milan, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurilio","family":"Sampaolesi","sequence":"additional","affiliation":[{"name":"Laboratory of Translational Cardiomyology, Stem Cell Interdepartmental Institute, KU Leuven, Herestraat 49 O&N1 bus 814, 3000 Leuven, Belgium."},{"name":"Human Anatomy, University of Pavia, Via Forlanini 8, 27100 Pavia, Italy."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","reference":[{"key":"2021042607062228000_R1","doi-asserted-by":"crossref","first-page":"2410","DOI":"10.1126\/science.1070104","article-title":"Correction of ADA-SCID by stem cell gene therapy combined with nonmyeloablative conditioning","volume":"296","author":"Aiuti","year":"2002","journal-title":"Science"},{"key":"2021042607062228000_R2","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1002\/mus.880120705","article-title":"Regeneration of skeletal muscle fibers from autologous satellite cells multiplied in vitro. 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