Titre :
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Muscle-specific micrornas (MIRNAS) in Duchenne muscular dystrophy (DMD)
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contenu dans :
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Auteurs :
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Congrès international de myologie 2008 (International Congress of Myology 2008; 26-30 mai 2008; Marseille, France) ;
Maciotta S ;
Meregalli M ;
Farini A ;
Belicchi M ;
Parolini D ;
Bresolin N ;
Torrente Y
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Type de document :
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Article
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Année de publication :
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2008
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Pages :
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p. 132
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Langues:
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Anglais
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Mots-clés :
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colloque
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dystrophie musculaire de Duchenne
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Résumé :
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DMD is caused by frameshift mutations in the gene encoding for dystrophin. These mutations are responsible for the loss of function of the dystrophin protein that leads to membrane destabilization and subsequent activation of pathophysiological processes. Clinically this situation leads to rapidly progressive and severe skeletal muscle weakness. Recently it has been discovered that a restricted group of microRNAs (miR-1, miR-133, miR-181 and miR-206) are muscle-specific and seem to be involved in in vitro-induced myogenesis. Moreover a recent work based on microRNA Array Analyses reported a disregulation in the expression levels of several miRNAs in 10 distinct forms of muscular dystrophies. These data suggest that some miRNAs may be involved in the pathogenesis of DMD phenotype. The aim of our study is to understand if there is a participation of these regulatory molecules in the pathogenesis of DMD. In order to verify this hypothesis, up to now we compared the expression levels of miR-1 and miR-133 by Northern Blotting and real-time-PCR between different muscle tissues of a dystrophic mouse model (MDX) and normal mouse (C57BL). We also evaluated by the same techniques the expression of these miRNAs in fetal and adult human muscle (MSH) from DMD and normal subjects. Our data evidence a higher expression of both miRNAs in MDX muscles and in MSH isolated from DMD patients. Further studies need to be done to better understand the biological function of the upregulation of these two miRNAs in pathological tissues.
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