The pathoetiology of neurofibromatosis 1.

  title={The pathoetiology of neurofibromatosis 1.},
  author={Eeva-Mari Jouhilahti and Sirkku A Peltonen and Anthony M. Heape and Juha M. Peltonen},
  journal={The American journal of pathology},
  volume={178 5},
Although a mutation in the NF1 gene is the only factor required to initiate the neurocutaneous-skeletal neurofibromatosis 1 (NF1) syndrome, the pathoetiology of the multiple manifestations of this disease in different organ systems seems increasingly complex. The wide spectrum of different clinical phenotypes and their development, severity, and prognosis seem to result from the cross talk between numerous cell types, cell signaling networks, and cell-extracellular matrix interactions. The bi… CONTINUE READING