The Inoue Lab studies how skeletal muscle maintains protein quality and how disruption of this process leads to protein aggregation and inherited muscle disease. Skeletal muscle fibers are long-lived cells that must withstand continuous mechanical and metabolic stress, making effective protein quality control particularly important. The lab uses genetically defined muscle diseases as models to understand the fundamental mechanisms of proteostasis failure.
A major focus of the lab is protein aggregate myopathies caused by defects in molecular chaperones, particularly DNAJB4-related myopathy. The team investigates how disease-causing variants alter chaperone function, client protein handling and cooperative chaperone networks, and how these defects lead to selective vulnerability of skeletal muscle. By combining cell and mouse models, human muscle pathology, molecular and cell biology, muscle physiology and proteomic approaches, the lab's long-term goal is to identify mechanism-based treatments for currently untreatable muscle diseases.
