Defining development factor requirements for progenitors facilitates their characterization and amplification. We characterize a peripheral nervous system embryonic dorsal rootTyrosine hydroxylase ganglion progenitor population using in vitro clonal sphere-formation assays. Cells differentiate into glial cells, smooth muscle/fibroblast (SM/Fb)-like cells, and neurons. Genetic never and pharmacologic equipment uncovered that sphere formation necessitates signaling in the EGFR tyrosine kinase. Nf1 reduction of perform amplifies this progenitor pool, which becomes hypersensitive to growth factors and confers tumorigenesis. DhhCre;Nf1(fl/fl) mouse neurofibromas consist of a progenitor population with equivalent development demands, possible, and marker expression. In people, NF1 mutation predisposes to benign neurofibromas, incurable peripheral nerve tumors.
Potential identification of human EGFR(+);P75(+) neurofibroma cells enriched EGF-dependent sphere-forming cells. Neurofibroma spheres include glial-like progenitors that differentiate into neurons and SM/Fb-like cells in vitro and kind benign neurofibroma-like lesions in nude mice. We suggest that expansion of an EGFR-expressing early glial progenitor contributes to neurofibroma formation.