FSHD myoblasts fail to downregulate intermediate filament protein vimentin during myogenic differentiation

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant hereditary neuromuscular disorder. The clinical features of FSHD include weakness of the facial and shoulder girdle muscles followed by wasting of skeletal muscles of the pelvic girdle and lower extremities. Although FSHD myoblas...

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Bibliographic Details
Published in:Biopolimery i kletka 2011, Vol.27 (5), p.359-363
Main Authors: Dmitriev, P. V., Barat, A. L., Cochet, E., Ogryzko, V. V., Laoudj-Chenivesse, D., Lipinski, M., Vassetzky, Y. S.
Format: Article
Language:English
Subjects:
Hereditary diseases
Muscular dystrophy
Myoblasts
Myotubes
Pelvis
Skeletal muscle
Vimentin
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Summary:Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant hereditary neuromuscular disorder. The clinical features of FSHD include weakness of the facial and shoulder girdle muscles followed by wasting of skeletal muscles of the pelvic girdle and lower extremities. Although FSHD myoblasts grown in vitro can be induced to differentiate into myotubes by serum starvation, the resulting FSHD myotubes have been shown previously to be morphologically abnormal.
ISSN:0233-7657
1993-6842
DOI:10.7124/bc.000123