Unravelling inclusion body myositis using a patient-derived fibroblast model.

Journal of cachexia, sarcopenia and muscle(2023)

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摘要
These findings confirm the presence of molecular disturbances in peripheral tissues from IBM patients and prompt patients' derived fibroblasts as a promising disease model, which may eventually be exported to other neuromuscular disorders. We additionally identify new molecular players in IBM associated with disease progression, setting the path to deepen in disease aetiology, in the identification of novel biomarkers or in the standardization of biomimetic platforms to assay new therapeutic strategies for preclinical studies.
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关键词
Autophagy,Fibroblasts,Inclusion body myositis,Inflammation,Mitochondria,Myopathy
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