Reviews
Disease models of myositis: overview of cell culture and rodent systems
M. Hauke1, M. Kamiya2, C.C. Weihl3, J. Schmidt4
- Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel University Hospital, Brandenburg Medical School Theodore Fontane, Rüdersdorf bei Berlin; and Brandenburg Medical School Theodor Fontane, Faculty of Health Sciences Brandenburg, Rüdersdorf bei Berlin, Germany.
- Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
- Department of Neurology, Washington University School of Medicine, Saint Louis, MO, USA.
- Department of Neurology and Pain Treatment, Neuromuscular Center, Center for Translational Medicine, Immanuel University Hospital, Brandenburg Medical School Theodore Fontane, Rüdersdorf bei Berlin; Brandenburg Medical School Theodor Fontane, Faculty of Health Sciences Brandenburg, Rüdersdorf bei Berlin; and Department of Neurology, Neuromuscular Center, University Medical Center Göttingen, Göttingen, Germany. j.schmidt@gmx.org
CER17256
2025 Vol.43, N°2
PI 0345, PF 0353
Reviews
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PMID: 39907590 [PubMed]
Received: 31/10/2023
Accepted : 20/05/2024
In Press: 03/02/2025
Published: 26/02/2025
Abstract
Inflammatory myopathies (in short: myositis) display a heterogenic group of rare inflammatory diseases of the skeletal muscle and other organs such as lung, heart and skin. Patients typically display muscular weakness, wasting and a variable response to treatment. The pathogenesis involves invasion of muscle fibres by mononuclear cells and deposition of autoantibodies. In vitro and in vivo models are crucial to understand the so far unresolved complex network of pathomechanisms and how to design future treatment strategies. So far, no model can represent all features of the human disease, but each facilitates analysis of distinct mechanisms of the disease. A range of different in vitro and in vivo models have been developed in recent years to functionally study myositis pathology. This review provides an overview of muscle cell culture systems and transgenic as well as inducible animal models that each represent distinct features of myositis.
