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Unacylated Ghrelin Enhances Satellite Cell Function and Relieves the Dystrophic Phenotype in Duchenne Muscular Dystrophy mdx Model
Author(s) -
Reano Simone,
Angelino Elia,
Ferrara Michele,
Malacarne Valeria,
Sustova Hana,
Sabry Omar,
Agosti Emanuela,
Clerici Sara,
Ruozi Giulia,
Zentilin Lorena,
Prodam Flavia,
Geuna Stefano,
Giacca Mauro,
Graziani Andrea,
Filigheddu Nicoletta
Publication year - 2017
Publication title -
stem cells
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.159
H-Index - 229
eISSN - 1549-4918
pISSN - 1066-5099
DOI - 10.1002/stem.2632
Subject(s) - duchenne muscular dystrophy , biology , regeneration (biology) , dystrophin , myocyte , muscular dystrophy , microbiology and biotechnology , muscle atrophy , fibrosis , endocrinology , mdx mouse , medicine , skeletal muscle , genetics
Muscle regeneration depends on satellite cells (SCs), quiescent precursors that, in consequence of injury or in pathological states such as muscular dystrophies, activate, proliferate, and differentiate to repair the damaged tissue. A subset of SCs undergoes self‐renewal, thus preserving the SC pool and its regenerative potential. Unacylated ghrelin (UnAG) is a circulating hormone that protects muscle from atrophy, promotes myoblast differentiation, and enhances ischemia‐induced muscle regeneration. Here we show that UnAG increases SC activity and stimulates Par polarity complex/p38‐mediated asymmetric division, fostering both SC self‐renewal and myoblast differentiation. Because of those activities on different steps of muscle regeneration, we hypothesized a beneficial effect of UnAG in mdx dystrophic mice, in which the absence of dystrophin leads to chronic muscle degeneration, defective muscle regeneration, fibrosis, and, at later stages of the pathology, SC pool exhaustion. Upregulation of UnAG levels in mdx mice reduces muscle degeneration, improves muscle function, and increases dystrophin‐null SC self‐renewal, maintaining the SC pool. Our results suggest that UnAG has significant therapeutic potential for preserving the muscles in dystrophies. S tem C ells 2017;35:1733–1746

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