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The biological function of the cellular prion protein: an update.

  • Wulf MA Institute of Neuropathology, University of Zurich, Rämistrasse 100, CH-8091, Zürich, Switzerland.
  • Senatore A Institute of Neuropathology, University of Zurich, Rämistrasse 100, CH-8091, Zürich, Switzerland.
  • Aguzzi A Institute of Neuropathology, University of Zurich, Rämistrasse 100, CH-8091, Zürich, Switzerland. adriano.aguzzi@usz.ch.
  • 2017-05-04
Published in:
  • BMC biology. - 2017
Animals
Central Nervous System
Disease Models, Animal
Mice
Peripheral Nervous System
Prion Diseases
Prion Proteins
English The misfolding of the cellular prion protein (PrPC) causes fatal neurodegenerative diseases. Yet PrPC is highly conserved in mammals, suggesting that it exerts beneficial functions preventing its evolutionary elimination. Ablation of PrPC in mice results in well-defined structural and functional alterations in the peripheral nervous system. Many additional phenotypes were ascribed to the lack of PrPC, but some of these were found to arise from genetic artifacts of the underlying mouse models. Here, we revisit the proposed physiological roles of PrPC in the central and peripheral nervous systems and highlight the need for their critical reassessment using new, rigorously controlled animal models.
Language
  • English
Open access status
gold
Identifiers
Persistent URL
https://sonar.ch/global/documents/47553
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