The heat shock response modulates transthyretin deposition in the peripheral and autonomic nervous systems.

@article{Santos2010TheHS,
  title={The heat shock response modulates transthyretin deposition in the peripheral and autonomic nervous systems.},
  author={Sofia Duque Santos and Rui D V Fernandes and Maria Jo{\~a}o Saraiva},
  journal={Neurobiology of aging},
  year={2010},
  volume={31 2},
  pages={280-9}
}
Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease that selectively affects the peripheral nervous system. The putative cause of this life threatening pathology is tissue deposition of mutant transthyretin (TTR), initially as non-fibrillar deposits and later as fibrillar material. The mouse models currently available do not recapitulate the human whole features, since the peripheral nervous tissue is spared. We have characterized a new mouse model expressing the human… CONTINUE READING
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