Prions and manganese: A maddening beast
1Department of Biology and Biochemistry, University of Bath, Bath, UKBA2 7AY. bssdrb@bath.ac.uk
Metallomics : Integrated Biometal Science
|March 11, 2011
Summary
Manganese may play a crucial role in prion diseases, potentially stabilizing prions and offering therapeutic targets. Chelation therapy targeting manganese shows promise in extending lifespan in affected animals.
Area of Science:
- Neurodegenerative diseases
- Prion biology
- Metal-protein interactions
Background:
- Prion diseases, such as variant Creutzfeldt-Jakob disease (vCJD), are transmissible neurodegenerative disorders.
- The prion protein (PrP) is known for its association with these diseases and its role as a copper-binding protein.
- The interaction of PrP with other metals, particularly manganese (Mn), has been historically underestimated compared to copper.
Purpose of the Study:
- To review the existing evidence linking manganese to prion diseases.
- To highlight the emerging role of manganese in prion stabilization and disease progression.
- To explore the therapeutic potential of targeting manganese in prion disease treatment.
Main Methods:
- Literature review of studies investigating the PrP-manganese interaction.
- Analysis of data on manganese's effect on prion stability in the environment.
- Examination of results from chelation therapy trials targeting manganese in animal models.
Main Results:
- Recent findings suggest manganese can stabilize prions, potentially contributing to their environmental persistence.
- Chelation therapy specifically targeting manganese has demonstrated an ability to extend survival times in animal models of prion disease.
- The historical underestimation of the manganese-prion link is being challenged by new data.
Conclusions:
- Manganese represents a significant, yet underappreciated, factor in prion disease pathogenesis.
- Targeting manganese through chelation therapy offers a promising new avenue for treating prion diseases.
- Further research into the PrP-manganese interaction is warranted to fully understand its implications for disease and therapy.
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