Altered distribution of lamin and emerin in muscle nuclei of sIBM patients

Clin Neuropathol. 2008 Nov-Dec;27(6):424-9. doi: 10.5414/npp27424.

Abstract

Objective: Sporadic inclusion body myositis (sIBM) is a chronic acquired inflammatory myopathy. The cause of sIBM remains unknown and its pathogenesis is controversial. There is a hypothesis [Karpati and Carpenter 1993] that the rimmed vacuoles result from nuclear breakdown, and IBM filaments are formed from components of the nuclear matrix.

Material and methods: For nuclear membrane protein detection, six IBM patients were studied using immunohistochemical and immunochemical techniques.

Results: It was demonstrated that in the interior of 10-15% myonuclei emerin and lamin A/C inclusions appeared constantly. This finding indicated an abberant localization of lamin A/C epitopes, the presence of presumptive lamin A (67 KDu) and emerin as in the affected nuclei.

Conclusion: We support the suggestion that truncated, changed lamin A protein takes part in nuclear disintegration and rimmed vacuole formation.

MeSH terms

  • Aged
  • Case-Control Studies
  • Cell Nucleus / metabolism*
  • Cohort Studies
  • Humans
  • Lamins / metabolism*
  • Membrane Proteins / metabolism*
  • Middle Aged
  • Myositis, Inclusion Body / etiology
  • Myositis, Inclusion Body / metabolism*
  • Myositis, Inclusion Body / pathology*
  • Nuclear Proteins / metabolism*
  • Quadriceps Muscle / metabolism*
  • Quadriceps Muscle / pathology

Substances

  • Lamins
  • Membrane Proteins
  • Nuclear Proteins
  • emerin