Trafficking of the cellular isoform of the prion protein

Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. Although the nature of the responsible agent of these diseases is uncertain, it is clear that a protein called PrP Sc has a ce...

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Bibliographic Details
Published in:Biomedicine & pharmacotherapy 1999, Vol.53 (1), p.39-46
Main Authors: Lehmann, S., Milhavet, O., Mangé, A.
Format: Article
Language:English
Subjects:
Animals
cellular trafficking
Endoplasmic Reticulum - chemistry
Endoplasmic Reticulum - ultrastructure
Humans
Isomerism
prion protein
PrPC Proteins - chemistry
PrPC Proteins - metabolism
transmissible spongiform encephalopathies
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Summary:Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. Although the nature of the responsible agent of these diseases is uncertain, it is clear that a protein called PrP Sc has a central role in their pathology. PrP Sc is a conformational variant of a normal protein called PrP C. Understanding the transition from PrP C to PrP Sc is a major issue in the field. In this article, we will review what is known about the cell biology of PrP C, the understanding of which is crucial considering that trafficking of this molecule governs generation of PrP Sc.
ISSN:0753-3322
1950-6007
DOI:10.1016/S0753-3322(99)80059-6