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Author Spotlight: Insight Into Advances in Prion Diseases Research
Published on: August 11, 2023
736
Recent developments in antibody therapeutics against prion disease
Karl Frontzek1, Adriano Aguzzi1
1Institute of Neuropathology, University of Zurich, Zurich, Switzerland.
Emerging Topics in Life Sciences
|July 8, 2020
Summary
Passive immunotherapy shows promise for prion diseases like Creutzfeldt-Jakob
Area of Science:
- Neuroscience
- Immunology
- Protein Chemistry
Background:
- Prion diseases, including Creutzfeldt-Jakob's disease (CJD), are fatal neurodegenerative disorders.
- Passive immunotherapy using antibodies against the cellular prion protein (PrPC) is a potential therapeutic strategy.
- Some anti-PrPC antibodies exhibit toxicity, linked to the protein's flexible amino-terminal tail.
Purpose of the Study:
- To provide a perspective on the therapeutic potential and challenges of anti-prion antibodies.
- To discuss the implications of recent clinical and epidemiological findings in anti-prion antibody research.
Main Methods:
- Review of preclinical evidence on passive immunotherapy for prion diseases.
- Analysis of structure-function relationships of PrPC and antibody interactions.
- Consideration of a first-in-human study of an anti-prion antibody in sporadic CJD patients.
- Evaluation of data from large-scale serosurveys on human anti-prion autoantibodies.
Main Results:
- Preclinical studies support passive immunotherapy for prion diseases.
- The flexible N-terminal tail of PrPC is crucial for antibody-mediated toxicity.
- A first-in-human trial for sporadic CJD using an anti-prion antibody has been initiated.
- Prevalence of natural human anti-prion autoantibodies has been mapped.
Conclusions:
- Therapeutic anti-prion antibodies offer opportunities but face limitations.
- Understanding PrPC structure-function is key to developing safe and effective antibody therapies.
- Further research is needed to optimize passive immunotherapy for human prion diseases.
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