Open AccessSkeletal Muscle Differentiation on a Chip Shows Human Donor Mesoangioblasts’ Efficiency in Restoring Dystrophin in a Duchenne Muscular Dystrophy Model
Author(s) -
Serena Elena,
Zatti Susi,
Zoso Alice,
Lo Verso Francesca,
Tedesco F. Saverio,
Cossu Giulio,
Elvassore Nicola
Publication year - 2016
Publication title -
stem cells translational medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.781
H-Index - 71
eISSN - 2157-6580
pISSN - 2157-6564
DOI - 10.5966/sctm.2015-0053
Subject(s) - dystrophin , duchenne muscular dystrophy , myogenesis , myocyte , utrophin , muscular dystrophy , progenitor cell , skeletal muscle , microbiology and biotechnology , medicine , biology , stem cell
A microengineered model was used to provide in vitro quantitative evidence of the ability of mesoangioblasts to restore dystrophin, in terms of protein accumulation and distribution, within myotubes derived from patients with Duchenne muscular dystrophy (DMD). Results showed healthy myoblasts and mesoangioblasts restored dystrophin expression in DMD myotubes. However, mesoangioblasts showed unexpected efficiency with respect to myoblasts in dystrophin production and length of the dystrophin membrane domain.