As the proteasome inhibitor BTZ affects the mitochondria promoting generation of reactive oxygen species (ROS), alteration in the mitochondrial membrane potential and release of cytochrome c [29], we hypothesized that MM cells may activate TLR4 signalling during exposure to BTZ as a stress-responsive mechanism to protect mitochondria against drug-induced apoptosis. The gene discussed is TLR4; the disease is Miyoshi myopathy.