Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases
2008
TDP-43 Pathology in Human Prion Diseases
publication
Evidence: moderate
Author Information
Author(s): Isaacs A M, Powell C, Webb T E, Linehan J M, Collinge J, Brandner S
Primary Institution: MRC Prion Unit, UCL Institute of Neurology
Hypothesis
Is there TDP-43 pathology in human prion diseases?
Conclusion
Human prion diseases do not exhibit detectable TDP-43 pathology.
Supporting Evidence
- TDP-43 did not co-localize with ubiquitin-positive PrP plaques or diffuse PrP aggregates.
- Most PrP plaques contained ubiquitin, while synaptic PrP deposits were not associated with ubiquitin.
- The lack of TDP-43 pathology suggests it is not involved in prion disease pathogenesis.
Takeaway
This study found that a protein called TDP-43, which is linked to some brain diseases, is not present in prion diseases, suggesting they are different types of brain problems.
Methodology
Immunohistochemistry and double-labelling immunofluorescence were used to analyze TDP-43, ubiquitin, and PrP in various forms of human prion disease.
Limitations
The study only examined a limited number of cases and did not explore all potential prion disease variants.
Digital Object Identifier (DOI)
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