Boundary cap cells in development and disease

Broad plasticity of the peripheral glia is an emerging concept during development of the peripheral nervous system (PNS). Recent studies have identified the neural crest-derived boundary caps (BCs), as a multitask stem cell population of the developing PNS. BC progeny migrate along the nerves to provide the major glial component of nerve roots and nerve terminals in the skin. Strikingly, those two locations constitute the privileged sites for development of benign peripheral nerve sheath tumors called neurofibromas in patients with neurofibromatosis type 1 (NF1), making BCs attractive candidates for the 'cell of origin' of this disease. Here, we review these exciting findings, focusing on the origin and novel functions of BCs. We further discuss the heterogeneity of BCs, and address their implication in the pathogenesis of NF1.