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Oxidative stress in desminopathies and myotilinopathies: a link between oxidative damage and abnormal protein

Anna Janué1, Montse Olivé, Isidre Ferrer

  • 1Institut de Neuropatologia, Servei Anatomia Patològica, IDIBELL-Hospital Universitari de Bellvitge CIBERNED, Hospitalet de Llobregat, Barcelona, Spain.

Brain Pathology (Zurich, Switzerland)
|September 6, 2007
PubMed

Insights

Oxidative stress markers are elevated in myopathies like myotilinopathies and desminopathies. This study links oxidative stress to protein aggregation in these muscle diseases.

Area of Science:

  • Muscle Biology
  • Molecular Medicine
  • Pathology

Background:

  • Myotilinopathies and desminopathies are myofibrillar myopathies (MFM) characterized by protein aggregates in muscle cells.
  • Protein oxidation can promote aggregation and resistance to degradation, suggesting a role in MFM pathogenesis.

Purpose of the Study:

  • To investigate the presence and extent of oxidative stress in MFM, specifically myotilinopathies and desminopathies.
  • To analyze markers of glycoxidation, lipoxidation, and nitration in patient muscle biopsies.

Main Methods:

  • Analysis of muscle biopsies from patients with myotilinopathy and desminopathy.
  • Utilized gel electrophoresis, Western blotting, immunohistochemistry, and confocal microscopy.
  • Examined markers including AGEs, CML, CEL, MDAL, HNE, nitrotyrosine, NOS isoforms, and SOD2.

Main Results:

  • Elevated levels of advanced glycation end products (AGEs), CML, CEL, MDAL, HNE, and nitrotyrosine were observed in MFM.
  • Aberrant expression of these oxidative stress markers and related enzymes (NOS, SOD2) correlated with protein aggregates.
  • Co-localization of AGE, ubiquitin, and p62 was noted in affected muscle fibers.

Conclusions:

  • The study provides evidence linking oxidative stress to protein aggregation and abnormal protein deposition in myotilinopathies and desminopathies.
  • Oxidized proteins may contribute to MFM pathology by promoting misfolding and evading the ubiquitin-proteasome system (UPS).

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