Muscular dystrophies are caused by mutations in various genes but all MD patients experience a shared muscle pathology known as muscle fatty degeneration (FD). Muscle FD occurs when muscle fibers necrotize and are replaced by adipocytes and fibrotic tissue. As muscle is replaced with fat and scar tissue, the function and strength of those muscles decrease. The presence of muscle FD has been particularly well-documented in Limb Girdle Muscular Dystrophy 2B (LGMD2B) patients in natural history studies using MRI scans and muscle biopsies. LGMD2B is caused by mutations in dysferlin and initially attacks shoulder and hip muscles but eventually affects muscles in the legs and arms as well, which typically causes patients to be wheelchair-bound by age thirty. There is currently no cure or treatment for LGMD2B beyond PT and supportive devices. Glucocorticoids, which are the standard-of-care for many MDs, worsen the progression of LGMD2B and are not indicated. We are developing Advertent Biotherapeutic’s proprietary agent ADA011 into a therapy that inhibits muscle FD and preserves muscle function in LGMD2B patients. We identified ADA011 as a potent inhibitor of de novo adipogenesis in vitro and have now demonstrated that ADA011 inhibits FD and preserves muscle strength in a mouse model of LGMD2B (i.e., the Bla/J mice). In addition, we showed that ADA011 has favorable pharmacokinetic properties and is safe in mice providing a foundation for the clinical development of the agent.