Abstract
Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are fatal neurodegenerative disorders. An abnormal isoform of the prion protein (PrPSc) generated by post-translational modification of the cellular prion protein (PrPC) is believed to be the main component of this infectious agent. PrPSc is relatively resistant to proteinase K (PK) digestion. This characteristic has been widely accepted as the physicochemical basis for distinguishing between PrPC and PrPSc. PrPC is a glycoprotein that contains 2 Asnlinked glycosylation sites; it is present in the cells in 3 different glycoforms, including an unglycosylated form. Hence, for different prion strains, PrPSc exhibits different glycoform patterns with different ratios of the 3 forms by western blot. Recently, phenotypes of TSEs have emerged that exhibit PrPSc with different glycoform patterns and/or mild PK resistance in comparison with previously described typical cases. Regarding sheep scrapie, atypical scrapie cases that are represented by Nor98 have been reported among sheep previously presumed to be genetically scrapie-resistant. Moreover, atypical bovine spongiform encephalopathy (BSE) cases have been reported. These are classified into 2 phenotypes (H-type and L-type) based on the molecular weight of unglycosylated band of PK-digested PrPSc. The origin of these emerging prion diseases is obscure, conformational differences of PrPSc may cause the different biological and biochemical characteristics of prion strains.
Current Medicinal Chemistry
Title: Prion Diseases and Emerging Prion Diseases
Volume: 15 Issue: 9
Author(s): Takashi Yokoyama and Shirou Mohri
Affiliation:
Abstract: Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are fatal neurodegenerative disorders. An abnormal isoform of the prion protein (PrPSc) generated by post-translational modification of the cellular prion protein (PrPC) is believed to be the main component of this infectious agent. PrPSc is relatively resistant to proteinase K (PK) digestion. This characteristic has been widely accepted as the physicochemical basis for distinguishing between PrPC and PrPSc. PrPC is a glycoprotein that contains 2 Asnlinked glycosylation sites; it is present in the cells in 3 different glycoforms, including an unglycosylated form. Hence, for different prion strains, PrPSc exhibits different glycoform patterns with different ratios of the 3 forms by western blot. Recently, phenotypes of TSEs have emerged that exhibit PrPSc with different glycoform patterns and/or mild PK resistance in comparison with previously described typical cases. Regarding sheep scrapie, atypical scrapie cases that are represented by Nor98 have been reported among sheep previously presumed to be genetically scrapie-resistant. Moreover, atypical bovine spongiform encephalopathy (BSE) cases have been reported. These are classified into 2 phenotypes (H-type and L-type) based on the molecular weight of unglycosylated band of PK-digested PrPSc. The origin of these emerging prion diseases is obscure, conformational differences of PrPSc may cause the different biological and biochemical characteristics of prion strains.
Export Options
About this article
Cite this article as:
Yokoyama Takashi and Mohri Shirou, Prion Diseases and Emerging Prion Diseases, Current Medicinal Chemistry 2008; 15 (9) . https://dx.doi.org/10.2174/092986708783955437
DOI https://dx.doi.org/10.2174/092986708783955437 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
Call for Papers in Thematic Issues
Advances in Medicinal Chemistry: From Cancer to Chronic Diseases.
The broad spectrum of the issue will provide a comprehensive overview of emerging trends, novel therapeutic interventions, and translational insights that impact modern medicine. The primary focus will be diseases of global concern, including cancer, chronic pain, metabolic disorders, and autoimmune conditions, providing a broad overview of the advancements in ...read more
Approaches to the treatment of chronic inflammation
Chronic inflammation is a hallmark of numerous diseases, significantly impacting global health. Although chronic inflammation is a hot topic, not much has been written about approaches to its treatment. This thematic issue aims to showcase the latest advancements in chronic inflammation treatment and foster discussion on future directions in this ...read more
Cellular and Molecular Mechanisms of Non-Infectious Inflammatory Diseases: Focus on Clinical Implications
The Special Issue covers the results of the studies on cellular and molecular mechanisms of non-infectious inflammatory diseases, in particular, autoimmune rheumatic diseases, atherosclerotic cardiovascular disease and other age-related disorders such as type II diabetes, cancer, neurodegenerative disorders, etc. Review and research articles as well as methodology papers that summarize ...read more
Chalcogen-modified nucleic acid analogues
Chalcogen-modified nucleosides, nucleotides and oligonucleotides have been of great interest to scientific research for many years. The replacement of oxygen in the nucleobase, sugar or phosphate backbone by chalcogen atoms (sulfur, selenium, tellurium) gives these biomolecules unique properties resulting from their altered physical and chemical properties. The continuing interest in ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Potential Targets for the Development of Novel Antidepressants: Future Perspectives
CNS & Neurological Disorders - Drug Targets Skeletal Muscle in Motor Neuron Diseases: Therapeutic Target and Delivery Route for Potential Treatments
Current Drug Targets Allosteric Modulation of G Protein-Coupled Receptors
Current Pharmaceutical Design Metabotropic Glutamate Receptors (mGluRs) and Diabetic Neuropathy
Current Drug Targets Phytochemicals for Drug Discovery in Alzheimer’s Disease: <i>In Silico</i> Advances
Current Pharmaceutical Design GluN2B/N-methyl-D-aspartate Receptor Antagonists: Advances in Design, Synthesis, and Pharmacological Evaluation Studies
CNS & Neurological Disorders - Drug Targets Neuroinflammation and Alzheimer's Disease: Implications for Microglial Activation
Current Alzheimer Research The Protective Effects of Crocetin on Aβ<sub>1-42</sub>-Induced Toxicity in Ht22 Cells
CNS & Neurological Disorders - Drug Targets Novel Insights for Multiple Sclerosis and Demyelinating Disorders with Apoptosis, Autophagy, FoxO, and mTOR
Current Neurovascular Research Conference Report: 10th International Summer School of Neurology: Celebrating a Decade of Success: 5th July, 2015 – 9th July, 2015 | Eforie Nord, Romania
CNS & Neurological Disorders - Drug Targets Cannabinoid-related Agents in the Treatment of Anxiety Disorders: Current Knowledge and Future Perspectives
Recent Patents on CNS Drug Discovery (Discontinued) Sirtuins: Possible Clinical Implications in Cardio and Cerebrovascular Diseases
Current Drug Targets Pharmacophore, Similarity and ADMET Screening of Casein Kinase 1 inhibitors in Alzheimer's Disease
Current Bioactive Compounds Preface
Central Nervous System Agents in Medicinal Chemistry Midkine and Pleiotrophin in the Treatment of Neurodegenerative Diseases and Drug Addiction
Recent Patents on CNS Drug Discovery (Discontinued) Blood-brain Barrier Drug Discovery for Central Nervous System Infections
Current Drug Targets - Infectious Disorders Therapeutical Approaches of Vasoactive Intestinal Peptide as a Pleiotropic Immunomodulator
Current Pharmaceutical Design Oral Agents in Multiple Sclerosis
Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry An Update of the Classical and Novel Methods Used for Measuring Fast Neurotransmitters During Normal and Brain Altered Function
Current Neuropharmacology Effects of Anesthetics on Mitochondrial Signaling and Function
Current Drug Safety