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Updated: Jun 8, 2026
Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
[Muscular disability and organ impairments in myotonic dystrophy type 1]
1Médecine interne orientée vers les maladies orphelines et systémiques, CHU de Nancy, hôpitaux de Brabois, rue du Morvan, 54511 Vandœuvre cedex, France.
Background:
Myotonic dystrophy (DM1) is a multisystemic disorder characterized by myotonic muscular weakness, and numerous organ impairments, especially cardiac and respiratory disorders. The goal of this study was to evaluate in DM1 patients the relationships between a new muscular disability scale, the motor function measure (MFM), and functional measurements of organ involvements.
Patients And Methods:
The MFM and MDRS, as well as spirometry, blood gases, echocardiography, electrocardiogram, and ophthalomological examination were performed in 69 consecutive DM1 patients.
Results:
Significant relationships were found between MDRS and MFM (p < 0.001) as well as between both and age and BMI of the patients. Patients with total MFM below 76 had a higher risk of respiratory insufficiency, conduction disorders, left ventricular dysfunction, hypoxemia and cataract than the other DM1 patients.
Conclusion:
The MFM provides an effective evaluation of muscular disability in DM1 patients. Cardiac and respiratory involvements are correlated with lower MFM.
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