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An international research team with participation of researchers from the University Medical Center Göttingen (UMG), Germany, has developed a mouse model that offers new insights into the development of a rare but serious muscle disease. The researchers were able to demonstrate that the persistent inflammation in inclusion body myositis is linked to impaired cellular “garbage disposal” — an important clue for new treatment strategies. The findings have been published in the journal Brain.
Inclusion body myositis is a rare, chronic and progressive muscle disease that primarily affects people over the age of 45 and leads to muscle weakness and wasting. Proteins accumulate in the muscle fibers and cause changes in the mitochondria — the “powerhouses of the cells”. These mitochondria produce the energy needed to perform movements, for example. The thigh muscles and finger flexors are frequently affected by this disease, making everyday movements such as walking or grasping increasingly difficult. What makes this disease unique is that it responds barely to the standard anti-inflammatory medications that are successfully used for other forms of muscle inflammation.
In an international collaboration involving the Department of Neurology and the Department of Neuroimmunology and Multiple Sclerosis Research at the University Medical Center Göttingen (UMG), scientists have developed a mouse model for studying inclusion body myositis. The animal model provides new insights into the development of this rare but serious muscle disease, the cause of which has not yet been fully elucidated. Together with colleagues from Heidelberg, Aachen, Freiburg, Munich, and Switzerland, the researchers were able to show that chronic inflammation is linked to a disrupted “garbage disposal” system in the body’s cells. Both processes — inflammation and the disrupted “garbage disposal” — reinforce each other and jointly drive the disease.
New Approaches for Effective Treatment Strategies
“This could explain why traditional anti-inflammatory therapies fail and open up new avenues for developing effective treatment strategies,” says study co-author Priv.-Doz. Dr. Jana Zschüntzsch, senior physician in the Department of Neurology at UMG. “For the first time, our mouse model replicates all the key features of human inclusion body myositis. This is a huge step forward, because until now we lacked a suitable animal model to test new therapies.” Prof. Dr. Jens Schmidt, last author and specialist at the Department of Neurology at UMG, adds: “For cells to function, a cleaning system ensures that defective proteins and other cellular waste are properly disposed of. In our mouse model, this system is disrupted, while at the same time chronic inflammation persists — as if one were constantly stirring up dust in a cluttered household.”
The study is published in the journal Brain:
Original publication:
Bremer et al. Mutual reinforcement of lymphotoxin-driven myositis and impaired autophagy in murine muscle. Brain (2025). DOI: https://doi.org/10.1093/brain/awaf260
Mouse model enables investigation of causes
The researchers developed mice that were genetically modified to mimic both the inflammatory processes and the impaired cellular clearance associated with the human disease. These mice exhibited symptoms similar to those of patients suffering from inclusion body myositis.
A particularly important finding of the study: The researchers were able to demonstrate why standard anti-inflammatory drugs are ineffective in inclusion body myositis. Even when these drugs successfully suppressed the inflammation, muscle weakness and the characteristic cellular changes persisted. This reflects clinical experience, where steroids and other immunosuppressive drugs typically yield only disappointing results in patients.
In the coming years, the scientists will test various innovative therapeutic approaches.
Priv.-Doz. Dr. Jana Zschüntzsch, Department of Neurology, Phone +49 551 / 39-62520, j.zschuentzsch@med.uni-goettingen.de
Bremer et al. Mutual reinforcement of lymphotoxin-driven myositis and impaired autophagy in murine muscle. Brain (2025). DOI: https://doi.org/10.1093/brain/awaf260
Illustrative image.
Copyright: umg/swen pförtner
Priv.-Doz. Dr. Jana Zschüntzsch, senior physician at the Department of Neurology at the University M ...
Copyright: umg/frank stefan kimmel
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Illustrative image.
Copyright: umg/swen pförtner
Priv.-Doz. Dr. Jana Zschüntzsch, senior physician at the Department of Neurology at the University M ...
Copyright: umg/frank stefan kimmel
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