Correction of the FSHD myoblast differentiation defect by fusion with healthy myoblasts

ABSTRACT: Facioscapulohumeral dystrophy (FSHD) is a neuromuscular disease characterized by progressive asymmetric muscle weakness. Myoblasts isolated from FSHD muscles exhibit morphological differentiation defects and show a distinct transcription profile. These abnormalities may be linked to the muscle weakness in FSHD patients. Here, we have tested whether fusion of FSHD myoblasts (obtained from 2 patients) with primary myoblasts isolated from 2 healthy individuals could correct the differentiation defects. Our results show that the number of hybrid myotubes with normal phenotype increased with the percentage of normal myoblasts initially cultured. We demonstrated that a minimum of 50% of normal nuclei is required for a phenotypic correction of the FSHD phenotype. To test the correction on the fu

ORGANISM(S): Homo sapiens

SUBMITTER: Dessen Philippe 

PROVIDER: E-MTAB-3658 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress