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Updated: Aug 12, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Desmin phosphorylation abnormalities in cytoplasmic body and desmin-related myopathies
1Laboratory of Neuropathology, Centre Hospitalier Regional et Universitaire de Caen, Côte de Nacre, F-14033 Caen, France.
Abstract:
Cytoplasmic body myopathy (CBM) and desmin-related myopathy (DRM) are both characterized by an abnormal accumulation of desmin. To determine whether these abnormalities involve similar or different forms of desmin, we performed desmin two-dimensional electrophoresis: our results showed an increase in the two acidic isoforms in CBM muscles as compared with an increase in the number of acidic isovariants in DRM samples. A process of hyperphosphorylation involved in these acidic forms was confirmed by alkaline phosphatase application onto the muscle samples in both pathological conditions.
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