Project description:Fibrodysplasia ossificans progressiva (FOP) is a ultra rare disease causing the formation of extraskeletal bone in skeletal muscles and joint areas of affected patients, leading to progressive invalidation. To model the disease, we used the inducible Acvr1R206HloxP/loxP:CreERT2 mouse model. This study aims at investigating the early events occurring in FOP muscles, with a focus on unraveling if and how the interactions between macrophages and fibroadipogenic progenitors (FAPs) drive the formation of ectopic bone.
Project description:We compared the transcriptome of sorted muscle stem/progenitor cells differentiated from control and FOP (ACVR1 R206H (c.617>A)) human induced pluripotent stem cells.