Abstract
Prion diseases are rare fatal neurodegenerative disorders that may either occur sporadically, or be inherited or infectiously acquired in humans. Irrespective of etiology, they can be transmitted to other individuals, this fact being responsible for the public attention prion diseases have received especially since the nineteen nineties, when a new variant of Creutzfeldt-Jakob disease linked to the consumption of prion contaminated beef occurred for the first time in Great Britain. The infectious particle, termed prion, is presumably composed exclusively of a misfolded, partially proteaseresistant conformer (PrPSc) of a normal cell surface protein, the cellular prion protein (PrPC). The pathogenesis of prion diseases comprises entry, spread, and amplification of infectivity in the body periphery in infectiously acquired forms, as well as mechanisms of neuronal cell death in the central nervous system in all disease subtypes. Most experimental therapeutic approaches are either targeted to PrPC or PrPSc, or to the process of conversion from PrPC to PrPSc. Neuroprotective strategies aiming at an interruption of central nervous system pathogenesis have also been tested, albeit with only moderate success. In this review, we discuss actual and potential drug targets in the context of the pathogenic mechanisms of prion diseases.
Keywords: Prions, Creutzfeldt-Jakob disease, scrapie, drug targets, therapy, pathogenesis, stress, neurodegeneration
CNS & Neurological Disorders - Drug Targets
Title: The Pathogenic Mechanisms of Prion Diseases
Volume: 6 Issue: 6
Author(s): T. Voigtlander and U. Unterberger
Affiliation:
Keywords: Prions, Creutzfeldt-Jakob disease, scrapie, drug targets, therapy, pathogenesis, stress, neurodegeneration
Abstract: Prion diseases are rare fatal neurodegenerative disorders that may either occur sporadically, or be inherited or infectiously acquired in humans. Irrespective of etiology, they can be transmitted to other individuals, this fact being responsible for the public attention prion diseases have received especially since the nineteen nineties, when a new variant of Creutzfeldt-Jakob disease linked to the consumption of prion contaminated beef occurred for the first time in Great Britain. The infectious particle, termed prion, is presumably composed exclusively of a misfolded, partially proteaseresistant conformer (PrPSc) of a normal cell surface protein, the cellular prion protein (PrPC). The pathogenesis of prion diseases comprises entry, spread, and amplification of infectivity in the body periphery in infectiously acquired forms, as well as mechanisms of neuronal cell death in the central nervous system in all disease subtypes. Most experimental therapeutic approaches are either targeted to PrPC or PrPSc, or to the process of conversion from PrPC to PrPSc. Neuroprotective strategies aiming at an interruption of central nervous system pathogenesis have also been tested, albeit with only moderate success. In this review, we discuss actual and potential drug targets in the context of the pathogenic mechanisms of prion diseases.
Export Options
About this article
Cite this article as:
Voigtlander T. and Unterberger U., The Pathogenic Mechanisms of Prion Diseases, CNS & Neurological Disorders - Drug Targets 2007; 6 (6) . https://dx.doi.org/10.2174/187152707783399175
DOI https://dx.doi.org/10.2174/187152707783399175 |
Print ISSN 1871-5273 |
Publisher Name Bentham Science Publisher |
Online ISSN 1996-3181 |
Call for Papers in Thematic Issues
Trends and perspectives in the rational management of CNS disorders
Central nervous system (CNS) diseases enforce a significant global health burden, driving ongoing efforts to improve our understanding and effectiveness of therapy. This issue investigates current advances in the discipline, focusing on the understanding as well as therapeutic handling of various CNS diseases. The issue covers a variety of diseases, ...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
Related Articles
-
Novel Detection System for Plant Protein Production of Pharmaceuticals and Impact on Conformational Diseases
Protein & Peptide Letters Conformational Diseases: Structural Studies of Aggregation of Polyglutamine Proteins
Current Computer-Aided Drug Design Melatonin-mitochondria Interplay in Health and Disease
Current Topics in Medicinal Chemistry Current Progresses on Nanodelivery Systems for the Treatment of Neuropsychiatric Diseases: Alzheimer’s and Schizophrenia
Current Pharmaceutical Design A Role for Calcineurin in Alzheimers Disease
Current Neuropharmacology Editorial (Thematic Issue: NAD<sup>+</sup> Biosynthesis and Signaling as an Emerging Area in Medicinal Chemistry)
Current Topics in Medicinal Chemistry C-Abl Inhibition; A Novel Therapeutic Target for Parkinson's Disease
CNS & Neurological Disorders - Drug Targets Advances in Neuroimaging for HIV-1 Associated Neurological Dysfunction: Clues to the Diagnosis, Pathogenesis and Therapeutic Monitoring
Current HIV Research Recent Advances in the Development of Selective Ligands for the Cannabinoid CB2 Receptor
Current Topics in Medicinal Chemistry Catechol-O-methyltransferase, Cognition and Alzheimer's Disease
Current Alzheimer Research Neurorescue Activity, APP Regulation and Amyloid-β Peptide Reduction by Novel Multi-Functional Brain Permeable Iron- Chelating- Antioxidants,M-30 and Green Tea Polyphenol, EGCG
Current Alzheimer Research Comparative Evaluation of Ethanolic Extracts of Bacopa monnieri, Evolvulus alsinoides, Tinospora cordifolia and their Combinations on Cognitive Functions in Rats
Current Aging Science Ionotropic Glutamate Receptors & CNS Disorders
CNS & Neurological Disorders - Drug Targets Co-Enzyme Q10 to Treat Neurological Disorders: Basic Mechanisms, Clinical Outcomes, and Future Research Direction
CNS & Neurological Disorders - Drug Targets Antioxidants as a Potential Therapy Against Age-Related Neurodegenerative Diseases: Amyloid Beta Toxicity and Alzheimers Disease
Current Pharmaceutical Design Protein Kinase C Isozymes: Memory Therapeutic Potential
Current Drug Targets - CNS & Neurological Disorders Predicting Stability of Mild Cognitive Impairment (MCI): Findings of a Community Based Sample
Current Alzheimer Research Perspectives in Nanomedicine-Based Research Towards Cancer Therapies
Current Nanoscience Therapeutic Potential of Vasoactive Intestinal Peptide and its Receptors in Neurological Disorders
CNS & Neurological Disorders - Drug Targets The Role of the Brain Renin-Angiotensin System in Neurodegenerative Disorders
Current Alzheimer Research