Investigating satellite glial cell phenotypes and functions in the pathogenesis of Friedreich’s ataxia

A major feature of FA is dysfunction and degeneration of a type of neuron (called proprioceptor) involved in the control of balance and motor coordination. The consequences are early motor disorders which, after 10 to 20 years, lead to severely reduced mobility. Until now, research has focused on proprioceptiveor neurons. However, very recent studies suggest that other cell types, the satellite glial cells (SGCs) which intimately surround proprioceptor cell bodies, and the immune cells may both contribute to the dysfunction of these neurons. However, the mechanisms involved remain poorly understood. Based on Dr. Agulhon’s preliminary work and that of the literature, she hypothesized that SGCs (in collaboration with immune cells) contributes to proprioceptor pathogenesis and sensorimotor alterations in FA. By specifically inactivating the expression of the gene encoding frataxin selectively in proprioceptors, or in all other cell types, including SGCs and immune cells, this project will explore the relationship between SGCs, immune cells and proprioceptors. This project has profound implications for breaking through the limitation of FA treatment.