Welcome to the Myology Group The focus of the Myology Group is fundamental, mechanistic and translational approaches to skeletal muscle biology. A shared goal is to test how a mechanistic understanding can be applied to neuromuscular diseases. A major impetus for creating the Myology Group is to gather new talent to an well-equipped and academically stimulating environment with complementary interests and approaches. The Myology Group was established in January of 2006 with 2 founding senior groups and presently has 5 research teams in total. The Myology Group is affiliated with the Institut Myology and the creation of the unit was made possible by a partnership between ParisVI/UPMC, Inserm and AFM. Furthermore, the Myology Group is associated with IFR14. The team of Drs. Sassoon and Marazzi is focused on stem cells in postnatal skeletal muscle as well as the role of cell stress effectors upon muscle atrophy (muscle wasting). In particular, we have identified a role for the master tumor suppressor gene, p53, in governing muscle responses to inflammatory signals such as tumor necrosis factor-alpha (TNFa). A key regulator in muscle tissue stress responses is PW1, which is also a marker of pluripotent stem cells in muscle tissue. This dual identity suggests an interplay between stem cell stress and muscle homeostasis. We are also investigating the role of Wnt genes governing epithelial-mesenchymal interactions during development in steroid hormone responsive tissues such as the female reproductive tract and how de-regulation of these interactions leads to oncogenesis. Dr. Relaix's is interested in the molecular and cellular mechanisms of muscle cell specification using mouse models and in the function of the transcription factors Pax3 and Pax7 in these processes, as well as during development and oncogenesis. Dr. Gomes's is interested in understanding how the cytoskeleton regulates nuclear positioning and what is the role of nuclear positioning during cell migration and myofiber formation. They are also studying how mutations in proteins associated with muscular dystrophies interfere with nuclear position during myofiber formation. Dr. Ana Ferreiro is studying early-onset myopathies (EOM), which are heterogeneous, inherited muscle disorders presenting with muscle weakness from birth or early infancy. We focus on getting a better knowledge of the mechanisms leading from the genetic defects in EOM to the phenotypical consequences in patients, in order to develop pathophysiology-based therapeutic approaches, namely pharmacological therapies. |
