Desmin phosphorylation abnormalities in cytoplasmic body and desmin-related myopathies

Muscle Nerve. 1999 Aug;22(8):1122-5. doi: 10.1002/(sici)1097-4598(199908)22:8<1122::aid-mus17>3.0.co;2-t.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cytoplasm / pathology*
  • Desmin / analysis*
  • Electrophoresis, Gel, Two-Dimensional
  • Humans
  • Isoelectric Focusing
  • Muscles / pathology*
  • Muscular Diseases / pathology*

Substances

  • Desmin