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Purification of Hsp104, a Protein Disaggregase
Published on: September 30, 2011
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Complement Regulatory Protein Factor H Is a Soluble Prion Receptor That Potentiates Peripheral Prion Pathogenesis
Sarah J Kane1, Taylor K Farley1,2, Elizabeth O Gordon1
1Prion Research Center, Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523.
Journal of Immunology (Baltimore, Md. : 1950)
|October 27, 2017
Summary
Factor H (fH), a complement regulatory protein, delays prion disease progression in mice lacking its gene. This suggests fH plays a key role in prion accumulation, replication, and pathogenesis.
Area of Science:
- Neuroimmunology
- Prion Biology
- Complement System
Background:
- Complement proteins like C3, C1q, and CD21/35 exacerbate prion disease.
- These proteins facilitate prion uptake, trafficking, and retention in the lymphoreticular system.
- Factor H (fH) normally regulates complement activation by binding host molecules.
Purpose of the Study:
- To investigate the role of factor H (fH) in prion disease pathogenesis.
- To determine if fH influences prion accumulation, replication, and disease onset.
Main Methods:
- Transgenic mice lacking "Cfh" alleles were used to study prion disease.
- Biophysical interaction between purified fH and prion rods was analyzed.
- Prion deposition in the brains of infected mice was assessed.
Main Results:
- Mice lacking "Cfh" showed delayed peripheral prion accumulation, replication, and pathogenesis in a gene-dose-dependent manner.
- A direct biophysical interaction between fH and prion rods was confirmed.
- fH was found to influence prion deposition in the brain.
Conclusions:
- Factor H (fH) plays a significant role in modulating prion disease progression.
- The interplay between complement and prions involves complex regulation of prion handling by immune cells.
- Complement regulatory proteins represent a novel therapeutic target for prion diseases.
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