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Updated: Feb 19, 2026
Monitoring Immune Cells Trafficking Fluorescent Prion Rods Hours after Intraperitoneal Infection
Published on: November 19, 2010
Experimental transfusion of variant CJD-infected blood reveals previously uncharacterised prion disorder in mice and
Emmanuel E Comoy1, Jacqueline Mikol2, Nina Jaffré2,3
1CEA, Institut François Jacob, Université Paris-Saclay, 18 Route du Panorama, 92265, Fontenay-aux-Roses, France. emmanuel.comoy@cea.fr.
Abstract:
Exposure of human populations to bovine spongiform encephalopathy through contaminated food has resulted in <250 cases of variant Creutzfeldt-Jakob disease (vCJD). However, more than 99% of vCJD infections could have remained silent suggesting a long-term risk of secondary transmission particularly through blood. Here, we present experimental evidence that transfusion in mice and non-human primates of blood products from symptomatic and non-symptomatic infected donors induces not only vCJD, but also a different class of neurological impairments. These impairments can all be retransmitted to mice with a pathognomonic accumulation of abnormal prion protein, thus expanding the spectrum of known prion diseases. Our findings suggest that the intravenous route promotes propagation of masked prion variants according to different mechanisms involved in peripheral replication.
Insights
Blood transfusions may transmit hidden prion diseases beyond variant Creutzfeldt-Jakob disease (vCJD). Experimental evidence in primates and mice shows masked prion variants can cause neurological impairments and spread via the intravenous route.
Area of Science:
- Neuroscience
- Infectious Diseases
- Biomedical Research
Background:
- Bovine spongiform encephalopathy (BSE) exposure caused <250 variant Creutzfeldt-Jakob disease (vCJD) cases.
- Over 99% of vCJD infections may be silent, posing a long-term blood-borne transmission risk.
Purpose of the Study:
- To investigate the potential for blood products to transmit prion diseases beyond vCJD.
- To explore the mechanisms of prion propagation via the intravenous route.
Main Methods:
- Transfusion of blood products from symptomatic and asymptomatic infected donors to mice and non-human primates.
- Retransmission studies in mice to assess induced neurological impairments.
- Analysis of prion protein accumulation.
Main Results:
- Transfusion induced vCJD and a novel class of neurological impairments.
- These new impairments were retransmissible and characterized by abnormal prion protein accumulation.
- Findings suggest masked prion variants propagate via distinct peripheral replication mechanisms.
Conclusions:
- The intravenous route facilitates the spread of masked prion variants.
- Prion diseases present a broader spectrum of neurological impairments than previously recognized.
- Blood transfusion poses a significant risk for transmitting novel prion-related neurological disorders.
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