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Updated: Jan 29, 2026

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Association between TDP-43 and mitochondria in inclusion body myositis

Mikayla L Huntley1, Ju Gao1, Pichet Termsarasab1

  • 1Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

Laboratory Investigation; a Journal of Technical Methods and Pathology
|February 12, 2019
PubMed

Insights

Inclusion body myositis (IBM) involves TDP-43 protein aggregates and mitochondrial issues. This study reveals TDP-43 accumulation alongside mitochondrial dysfunction in IBM muscle fibers, suggesting a link to disease progression.

Area of Science:

  • Neuromuscular disorders
  • Mitochondrial biology
  • Proteinopathies

Background:

  • Inclusion body myositis (IBM) is a progressive muscle disorder affecting individuals over 50.
  • Pathological hallmarks include p62 aggregates and mislocalized TDP-43 in muscle fibers.
  • Mitochondria are implicated in TDP-43 neurotoxicity in other neurodegenerative diseases.

Purpose of the Study:

  • To investigate the association between TDP-43 and mitochondria in skeletal muscle from IBM patients.
  • To determine if TDP-43 accumulation correlates with mitochondrial abnormalities in IBM.

Main Methods:

  • Analysis of skeletal muscle biopsies from IBM patients and age-matched controls.
  • Immunohistochemistry to detect TDP-43, p62, and mitochondrial oxidative phosphorylation complexes.
  • Immunoblot analysis to quantify protein levels.

Main Results:

  • TDP-43, phosphorylated TDP-43, and p62 co-localized with mitochondrial oxidative phosphorylation complexes in IBM muscle fibers.
  • Increased levels of TDP-43, truncated TDP-43, phosphorylated TDP-43, and p62 were observed in IBM patients.
  • Decreased levels of key subunits of mitochondrial oxidative phosphorylation complexes I and III were found in IBM patients.

Conclusions:

  • This study provides the first evidence of a strong association between TDP-43 accumulation and mitochondria in degenerating muscle fibers in IBM.
  • This association may play a role in the development of mitochondrial dysfunction and pathological protein aggregation in IBM.
  • Further research into this mitochondrial-TDP-43 axis could reveal novel therapeutic targets for IBM.

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