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.
Caron
A
A
Laboratory of Neuropathology, Centre Hospitalier Regional et Universitaire de Caen, Côte de Nacre, F-14033 Caen, France.
Chapon
F
F
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