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Updated: Aug 7, 2026
11:29
Isolation of Soluble and Insoluble PrP Oligomers in the Normal Human Brain
Published on: October 3, 2012
Copper and prion disease
1Department of Biochemistry, Cambridge University, Cambridge, UK. drb33@cam.ac.uk
Brain Research Bulletin
|July 27, 2001
Summary
The prion protein, a cell surface glycoprotein in neurons, binds copper and influences brain copper metabolism. Prion diseases may arise from disruptions in this copper homeostasis.
Area of Science:
- Neuroscience
- Biochemistry
- Cell Biology
Background:
- The function of the prion protein, a neuronal cell surface glycoprotein, was previously unknown.
- Recent findings indicate the prion protein is a copper-binding protein.
Purpose of the Study:
- To investigate the role of the prion protein in normal brain copper metabolism.
- To explore the implications of prion protein function in prion diseases.
Main Methods:
- Investigated copper binding properties of the prion protein.
- Assessed the effect of prion protein expression on cellular copper uptake.
- Examined the prion protein's role in superoxide dismutase activity.
- Studied the conversion of functional prion protein to aggregated amyloid forms.
Main Results:
- The prion protein binds copper and plays a role in brain copper metabolism.
- Prion protein expression affects cellular copper uptake and enhances copper incorporation into superoxide dismutase.
- The prion protein exhibits superoxide dismutase activity.
- Conversion to aggregated amyloid forms may alter or abolish prion protein function.
Conclusions:
- Prion protein is crucial for normal brain copper metabolism.
- Prion diseases might stem from disturbances in brain copper homeostasis due to altered prion protein function.
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