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| Content Provider | IEEE Xplore Digital Library |
|---|---|
| Author | Wen-Quan Zou Jue Yuan |
| Copyright Year | 2010 |
| Description | Author affiliation: Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, 2085 Adelbert Road, Cleveland, OH 44106, USA (Wen-Quan Zou; Jue Yuan) |
| Abstract | Prion protein (PrP) is probably the only known infectious protein that possesses chameleon-like features, as reflected not only in its conformation but also in its function. Cellular PrP $(PrP^{C}),$ rich in α-helical structures, is believed to be functionally involved in neurotransmitter metabolism, immune cell activation, cell adhesion, signal transduction, copper metabolism, antioxidant activity, and programmed cell death. Its pathologic conformer $(PrP^{Sc}),$ rich in β-sheet structures, plays an essential role in the pathogenesis of prion diseases, a group of age-related transmissible neurodegenerative disorders which, as in Alzheimer's disease, inevitably attack human cognition. Histologically, PrP is highly concentrated in the central nervous system although it is also widely expressed in the peripheral tissues and organs at lower levels. Observations indicating that a pronounced cognitive deficit is often evident in the early stage of prion diseases strongly imply that PrP is associated with human cognition. Indeed, cognitive deficit is one of a few phenotypes identified in mice lacking PrP. This article highlights evidence of the involvement of $PrP^{C}$ in human cognition. In addition, it puts forth the hypothesis that two potential PrP-implicated pathways are operative in human cognition. |
| Starting Page | 53 |
| Ending Page | 60 |
| File Size | 132065 |
| Page Count | 8 |
| File Format | |
| ISBN | 9781424480418 |
| e-ISBN | 9781424480425 |
| DOI | 10.1109/COGINF.2010.5599805 |
| Language | English |
| Publisher | Institute of Electrical and Electronics Engineers, Inc. (IEEE) |
| Publisher Date | 2010-07-07 |
| Publisher Place | China |
| Access Restriction | Subscribed |
| Rights Holder | Institute of Electrical and Electronics Engineers, Inc. (IEEE) |
| Subject Keyword | Proteins Diseases Humans Mice Cognition Iron memory Prion protein prion diseases cognition cognitive deficit insoluble prion protein Alzheimer's disease dementia |
| Content Type | Text |
| Resource Type | Article |
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