In patients with myositis associated with anti-Ku autoantibodies, a rare inflammatory myopathy characterized by prominent upregulation of autophagy, myofiber necrosis, MHC class I and II positivity, and variable endomysial inflammation, conspicuous sarcoplasmic protein aggregates were found to be LMP7-positive [157]. The gene discussed is PSMB8; the disease is myopathy.