Abstract
Background: Sporadic inclusion body myositis (sIBM) is the commonest inflammatory myopathy among aged population. Muscle histology is characterized by a combination of inflammation including mononuclear cellular infiltrates into endomysium and non-necrotic myofibers and degeneration including sarcoplasmic aggregation of TAR DNA-binding protein of 43 kDa (TDP-43). Cylindromatosis (CYLD), which is originally identified as a tumor suppressor, is a deubiquitinase that negatively regulates activation of NF-κB pathway known for inflammation and innate immunity.
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