Iron Imbalance in Prion Diseases
Author Information
Author(s): Ajay Singh, Alfred Orina Isaac, Xiu Luo, Mohan Maradumane L., Mark L. Cohen, Fusong Chen, Qingzhong Kong, Jason Bartz, Neena Singh
Primary Institution: Case Western Reserve University
Hypothesis
Is there an imbalance of brain iron homeostasis in prion diseases that contributes to neurotoxicity?
Conclusion
Prion disease-affected brains show a state of iron deficiency despite increased total iron levels, likely due to sequestration in PrPSc-ferritin complexes.
Supporting Evidence
- Prion disease-affected brains show increased total iron and redox-active iron.
- Transferrin levels increase in diseased brains despite iron excess, indicating iron deficiency.
- Sequestration of iron in PrPSc-ferritin complexes leads to cellular iron deficiency.
Takeaway
In prion diseases, the brain has too much iron but the cells can't use it, which can make the brain sick.
Methodology
The study analyzed brain tissues from humans, hamsters, and mice infected with prion diseases, measuring iron levels and related proteins.
Potential Biases
Potential bias due to the selection of samples from specific disease stages.
Limitations
The study primarily focuses on end-stage disease samples, which may not represent earlier disease stages.
Participant Demographics
The study included human, hamster, and mouse brain samples, with a focus on sporadic Creutzfeldt-Jakob disease (sCJD) cases.
Statistical Information
P-Value
p<0.0001
Statistical Significance
p<0.0001
Digital Object Identifier (DOI)
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