Abstract: A transgenic non-human animal model for Neurofibromatosis type 1, wherein the Nf1 gene is specifically inactivated in BC cells and derivatives thereof. Also, an in vitro method of producing cutaneous and plexiform Neurofibromas (NFBs) and/or for studying the development and composition of plexiform NFBs, including culturing in vitro Prss56-expressing cells and-derivatives thereof obtained from the transgenic non-human animal model. Further, a method for screening a candidate compound for use as a drug to treat Neurofibromatosis type 1, cutaneous NFBs and/or plexiform NFBs including contacting the candidate compound Prss56-expressing cells and-derivatives thereof obtained from the transgenic non-human animal model or administering the candidate compound to the transgenic non-human animal model.

Abstract: The present invention discloses the identification and characterization of a novel mammalian pluripotent neural cell population. More particularly, the present invention relates to compositions and methods allowing the identification, monitoring, culture and differentiation of boundary cap cells and their progeny. The invention also relates to methods of screening compounds that alter the growth, migration and/or differentiation of these cells, as well as to methods of producing functional cells in vitro, ex vivo or in vivo using said pluripotent cells. The invention can be used to detect, diagnose, monitor and/or treat various pathological conditions in mammalian subjects, including nervous diseases such as neurodegenerative condition, demyelination, pain, etc. The invention can be used for tissue engineering, to produce various differentiated cell types by differentiation of said pluripotent cells under appropriate conditions.