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Cell death in prion disease

H A Kretzschmar1, A Giese, D R Brown

  • 1Institut für Neuropathologic, Universität Gottingen, Federal Republic of Germany.

Journal of Neural Transmission. Supplementum
|January 1, 1997
PubMed
Summary

Prion diseases cause neurodegeneration via apoptosis. A synthetic prion protein peptide is toxic to normal neurons, but not in PrP knockout models, indicating microglia

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Prion Disease Research

Background:

  • Prion diseases are fatal neurodegenerative disorders.
  • The exact mechanisms of prion disease pathogenesis, including nerve cell death, remain unclear.
  • Prions are infectious agents composed of misfolded host proteins.

Purpose of the Study:

  • To investigate the mechanisms of nerve cell death in prion diseases.
  • To explore the role of the prion protein (PrP) and microglia in neurodegeneration.
  • To establish cell culture models for studying prion disease pathogenesis.

Main Methods:

  • In situ end-labeling technique and electron microscopy in a mouse scrapie model (79A strain).
  • Cell culture using a synthetic prion protein peptide (PrP106-126) with normal and PrP knockout (PrP0/0) mouse-derived neurons.
  • Assessment of reactive oxygen species production by microglia.

Main Results:

  • Nerve cell death in the scrapie model occurs via apoptosis.
  • The synthetic PrP peptide (PrP106-126) induces toxicity in normal neurons but not in PrP knockout neurons.
  • Microglia contribute to the neurotoxic process by secreting reactive oxygen species.

Conclusions:

  • Nerve cell death in prion diseases involves apoptosis.
  • Prion protein itself, or fragments thereof, can be directly neurotoxic to normal neurons.
  • Microglial activation and reactive oxygen species production are critical components of prion-induced neurodegeneration.
  • Further in vivo studies are needed to confirm these cell culture findings.

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