Prion Protein Modulates Cellular Iron Uptake: A Novel Function with Implications for Prion Disease Pathogenesis
2009

Prion Protein and Iron Uptake

publication Evidence: moderate

Author Information

Author(s): Ajay Singh, Mohan Maradumane L., Isaac Alfred Orina, Luo Xiu, Petrak Jiri, Vyoral Daniel, Singh Neena

Primary Institution: The Department of Pathology, Case Western Reserve University

Hypothesis

Does prion protein (PrPC) mediate cellular iron uptake and transport?

Conclusion

PrPC increases cellular iron levels and influences the transport of iron to ferritin, while mutant forms of PrP alter this process differently.

Supporting Evidence

  • PrPC increases intracellular iron levels in neuroblastoma cells.
  • Mutant PrP forms show differential effects on cellular iron levels.
  • Stimulation of PrP endocytosis increases ferritin iron content.
  • Cross-linking of PrP decreases its endocytosis and has minimal effect on ferritin iron.

Takeaway

The prion protein helps cells take in iron, which is important for brain health, but some mutations can mess this up.

Methodology

The study used human neuroblastoma cells to assess the effects of PrPC and mutant PrP forms on cellular iron levels and transport mechanisms.

Limitations

The study does not fully elucidate the precise biochemical pathways of iron modulation by PrP.

Statistical Information

P-Value

p<0.001

Statistical Significance

p<0.001

Digital Object Identifier (DOI)

10.1371/journal.pone.0004468

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