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Prion-like transmission of protein aggregates in neurodegenerative diseases

Journal

NATURE REVIEWS MOLECULAR CELL BIOLOGY
Volume 11, Issue 4, Pages 301-307

Publisher

NATURE PUBLISHING GROUP
DOI: 10.1038/nrm2873

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Funding

  1. Human Frontier Science Program
  2. MJ Fox Foundation for Parkinson's Research
  3. Swedish Brain Foundation
  4. Swedish Parkinson Foundation
  5. Soderberg Foundation
  6. Swedish Research Council
  7. Huntington's disease Society of America Coalition for the Cure
  8. CHDI Foundation
  9. National Institute of Neurological Disease and Stroke
  10. Agence Nationale de la Recherche
  11. Centre National de la Recherche Scientifique

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Neurodegenerative diseases are commonly associated with the accumulation of intracellular or extracellular protein aggregates. Recent studies suggest that these aggregates are capable of crossing cellular membranes and can directly contribute to the propagation of neurodegenerative disease pathogenesis. We propose that, once initiated, neuropathological changes might spread in a 'prion-like' manner and that disease progression is associated with the intercellular transfer of pathogenic proteins. The transfer of naked infectious particles between cells could therefore be a target for new disease-modifying therapies.

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