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Axonal prion protein is required for peripheral myelin maintenance
Juliane Bremer1, Frank Baumann, Cinzia Tiberi
1Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland.
Nature Neuroscience
|January 26, 2010
Summary
Prion protein PrP(C) is crucial for peripheral nerve health. Its absence in neurons causes chronic demyelinating polyneuropathy, highlighting its role in myelin maintenance.
Area of Science:
- Neuroscience
- Cell Biology
- Genetics
Background:
- Peripheral nerve integrity depends on axon-Schwann cell communication.
- Signals regulating myelin maintenance are distinct from myelination signals and poorly understood.
Purpose of the Study:
- To investigate the role of the prion protein (PrP(C)) in peripheral nerve myelin maintenance.
- To identify the cellular and molecular mechanisms underlying PrP(C)-dependent myelin maintenance.
Main Methods:
- Generated and analyzed four independent mouse strains with targeted ablation of the prion protein PrP(C).
- Investigated the effects of PrP(C) depletion specifically in neurons versus Schwann cells.
- Utilized PrP(C) variants with altered proteolytic cleavage properties to assess functional requirements.
Main Results:
- Ablation of PrP(C) consistently triggered chronic demyelinating polyneuropathy (CDP) across multiple mouse strains.
- CDP resulted from PrP(C) depletion specifically in neurons, not Schwann cells.
- PrP(C) variants requiring proteolytic cleavage were protective, while non-cleavable or secreted forms were not.
Conclusions:
- Neuronal expression of PrP(C) is essential for maintaining peripheral nerve myelin.
- Regulated proteolytic cleavage of PrP(C) is a critical mechanism for its function in myelin maintenance.
- PrP(C) plays a vital, cell-autonomous role in supporting axonal integrity via myelin maintenance.
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