The events leading to the degradation of the endogenous PrPC (normal cellular prion protein) have been the subject of numerous studies. Two cleavage processes, α-cleavage and β-cleavage, are responsible for the main C- and N-terminal fragments produced from PrPC. Both cleavage processes occur within the N-terminus of PrPC, a region that is significant in terms of function. α-Cleavage, an enzymatic event that occurs at amino acid residues 110 and 111 on PrPC, interferes with the conversion of PrPC into the prion disease-associated isoform, PrPSc (abnormal disease-specific conformation of prion protein). This processing is seen as a positive event in terms of disease development. The study of β-cleavage has taken some surprising turns. β-Cleavage is brought about by ROS (reactive oxygen species). The C-terminal fragment produced, C2, may provide the seed for the abnormal conversion process, as it resembles in size the fragments isolated from prion-infected brains. There is, however, strong evidence that β-cleavage provides an essential process to reduce oxidative stress. β-Cleavage may act as a double-edged sword. By β-cleavage, PrPC may try to balance the ROS levels produced during prion infection, but the C2 produced may provide a PrPSc seed that maintains the prion conversion process.
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Conference Article|
July 20 2012
Prion processing: a double-edged sword?
Hilary E.M. McMahon
Hilary E.M. McMahon
1
1UCD School of Biomolecular and Biomedical Science, Conway Institute for Biomolecular and Biomedical Research University College Dublin, Belfield, Dublin 4, Ireland
1emailhilary.mcmahon@ucd.ie
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Publisher: Portland Press Ltd
Received:
February 01 2012
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2012 Biochemical Society
2012
Biochem Soc Trans (2012) 40 (4): 735–738.
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Received:
February 01 2012
Citation
Hilary E.M. McMahon; Prion processing: a double-edged sword?. Biochem Soc Trans 1 August 2012; 40 (4): 735–738. doi: https://doi.org/10.1042/BST20120031
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