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Synaptic Dysfunction in Prion Diseases : A Trafficking Problem?
Joint Authors
Senatore, Assunta
Chiesa, Roberto
Restelli, Elena
Source
International Journal of Cell Biology
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-15, 15 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-11-28
Country of Publication
Egypt
No. of Pages
15
Main Subjects
Abstract EN
Synaptic dysfunction is an important cause of neurological symptoms in prion diseases, a class of clinically heterogeneous neurodegenerative disorders caused by misfolding of the cellular prion protein (PrPC).
Experimental data suggest that accumulation of misfolded PrPC in the endoplasmic reticulum (ER) may be crucial in synaptic failure, possibly because of the activation of the translational repression pathway of the unfolded protein response.
Here, we report that this pathway is not operative in mouse models of genetic prion disease, consistent with our previous observation that ER stress is not involved.
Building on our recent finding that ER retention of mutant PrPC impairs the secretory trafficking of calcium channels essential for synaptic function, we propose a model of pathogenicity in which intracellular retention of misfolded PrPC results in loss of function or gain of toxicity of PrPC-interacting proteins.
This neurotoxic modality may also explain the phenotypic heterogeneity of prion diseases.
American Psychological Association (APA)
Senatore, Assunta& Restelli, Elena& Chiesa, Roberto. 2013. Synaptic Dysfunction in Prion Diseases : A Trafficking Problem?. International Journal of Cell Biology،Vol. 2013, no. 2013, pp.1-15.
https://search.emarefa.net/detail/BIM-480236
Modern Language Association (MLA)
Senatore, Assunta…[et al.]. Synaptic Dysfunction in Prion Diseases : A Trafficking Problem?. International Journal of Cell Biology No. 2013 (2013), pp.1-15.
https://search.emarefa.net/detail/BIM-480236
American Medical Association (AMA)
Senatore, Assunta& Restelli, Elena& Chiesa, Roberto. Synaptic Dysfunction in Prion Diseases : A Trafficking Problem?. International Journal of Cell Biology. 2013. Vol. 2013, no. 2013, pp.1-15.
https://search.emarefa.net/detail/BIM-480236
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-480236