Desmin phosphorylation abnormalities in cytoplasmic body and desmin-related myopathies.
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.
Laboratory of Neuropathology, Centre Hospitalier Regional et Universitaire de Caen, Côte de Nacre, F-14033 Caen, France.
A
A
Caron
F
F
Chapon
D003593:Cytoplasm / Q000473:pathology*; D003893:Desmin / Q000032:analysis*; D015180:Electrophoresis, Gel, Two-Dimensional; D006801:Humans; D007525:Isoelectric Focusing; D009132:Muscles / Q000473:pathology*; D009135:Muscular Diseases / Q000473:pathology*
D003893:Desmin