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Role of copper and manganese in prion disease progression

Gerda Mitteregger1, Stefan Korte, Mehdi Shakarami

  • 1Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Feodor-Lynen-Str. 23, D-81377 Munich, Germany.

Brain Research
|July 29, 2009
PubMed

Insights

Dietary copper levels impact prion disease progression. Low copper shortens survival time in scrapie-infected mice, while high copper delays onset and affects cellular prion protein (PrP(C)) levels and cleavage.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Prion Disease Research

Background:

  • Cellular prion protein (PrP(C)) is crucial in prion disease pathogenesis.
  • Alterations in brain copper and manganese levels are observed in prion diseases, but their role is unclear.

Purpose of the Study:

  • To investigate the role of copper and manganese in prion disease pathogenesis.
  • To determine the effect of dietary copper on prion disease progression and PrP(C) metabolism.

Main Methods:

  • Analysis of synaptosomes from scrapie-infected mice.
  • Dietary manipulation of copper and manganese levels in mice.
  • Assessment of PrP(C) levels, cleavage, and resistance to proteases.

Main Results:

  • Scrapie infection led to reduced copper and increased manganese in mouse synaptosomes.
  • Low copper diets shortened survival time; high copper diets delayed disease onset.
  • Enhanced copper diets increased full-length and misfolded PrP(C) levels.
  • A copper-rich/manganese-poor diet decreased PrP(C) cleavage in healthy mice.

Conclusions:

  • Brain copper content influences PrP(C) levels and cleavage properties.
  • Copper availability may affect PrP conversion by altering functional PrP(C) levels, impacting prion disease progression.

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