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PrP expression and replication by Schwann cells: implications in prion spreading
Jérôme Follet1, Catherine Lemaire-Vieille, Françoise Blanquet-Grossard
1Laboratoire de Physiopathologie des Encéphalopathies Spongiformes Transmissibles, C.N.R.S. IFR3-Institut de Biologie de Lille-Institut Pasteur de Lille, 59021 Lille Cedex, France.
Journal of Virology
|February 12, 2002
Summary
Schwann cells express PrP(c) and can be infected by prions. This peripheral glial cell line replicates PrP(Sc) and transmits prion disease, offering new insights into neuroinvasion.
Area of Science:
- Neuroscience
- Cell Biology
- Infectious Diseases
Background:
- Prion diseases involve the spread of misfolded prion proteins (PrPSc) from peripheral sites to the central nervous system (CNS).
- Direct neuroinvasion via peripheral nerves is a proposed mechanism, but axonal transport speeds do not fully explain the observed incubation periods.
- Schwann cells, peripheral nerve glial cells, are hypothesized to play a role in prion transport and replication.
Purpose of the Study:
- To investigate the role of Schwann cells in prion neuroinvasion.
- To determine if Schwann cells express the cellular prion protein (PrPc) and can support PrPSc conversion and replication.
Main Methods:
- In vivo analysis of PrPc localization in mouse sciatic nerve samples.
- In vitro studies using the MSC-80 Schwann cell line to assess PrPc expression and prion infection.
- Infection of the Schwann cell line with the Chandler prion strain and subsequent transmission studies in mice.
Main Results:
- PrPc was primarily localized to the cell membrane of Schwann cells in vivo.
- The MSC-80 Schwann cell line expressed PrPc at the cell membrane.
- In vitro infection of MSC-80 cells with the Chandler prion strain led to PrPSc production after 18 passages.
- Infected Schwann cells transmitted prion disease to mice, which exhibited typical prion disease symptoms.
Conclusions:
- Schwann cells express PrPc and can be infected with prions.
- This study provides the first evidence of PrPSc infection and replication in a peripheral glial cell line.
- Schwann cells are a potential key player in prion neuroinvasion from the periphery to the CNS.