NF1 and plexiform neurofibroma: However, despite the considerable amount of data generated on the biochemistry of neurofibromin (structure, functions, interactions, localization, and regulation by PTMs) leading to the identification of several therapeutic targets and recent approval of the first therapy (selumetinib) for inoperable plexiform neurofibroma associated with NF1 (by targeting MEK kinase) [162], we are still far from having a complete picture of the functions of NF1 and their regulation.