Neurodegeneration in familial amyloid polyneuropathy: from pathology to molecular signaling.

@article{Sousa2003NeurodegenerationIF,
  title={Neurodegeneration in familial amyloid polyneuropathy: from pathology to molecular signaling.},
  author={M{\'o}nica Mendes Sousa and Maria Jo{\~a}o Saraiva},
  journal={Progress in neurobiology},
  year={2003},
  volume={71 5},
  pages={385-400}
}
Familial amyloid polyneuropathy (FAP) is an autosomal dominant neurodegenerative disorder related to the systemic deposition of mutated transthyretin (TTR) amyloid fibrils, particularly in peripheral nervous system (PNS). TTR fibrils are diffusely distributed in the PNS of FAP patients, involving nerve trunks, plexuses and ganglia. In peripheral nerves, amyloid deposits are prominent in the endoneurium, near blood vessels, Schwann cells and collagen fibrils. Fiber degeneration is axonal… CONTINUE READING