Repression of the NF1 gene by Tax may expain the development of neurofibromas in human T-lymphotropic virus type 1 transgenic mice.

摘要: In familial neurofibromatosis type 1 (NF1), individuals with a germ line-transmitted NF1 mutation develop multiple neurofibromas. To explain the observation that transgenic mice expressing human T-lymphotropic virus (HTLV-1) tax gene under control of viral regulatory element also neurofibromas, we demonstrate Tax trans-regulator can functionally repress expression through cis-acting located immediately upstream its transcriptional start site, thereby allowing development benign neurofibromas without need for direct mutations in NF1. We propose such mechanism would suffice to epigenetically alter expression. The fact animals have localized rather than diffuse neurofibroma formation, however, suggests additional genetic or epigenetic events may be required formation.