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
Diagnosis and treatment of central nervous system infectious diseases
Infectious diseases of the central nervous system (CNS) can be divided into bacterial, tuberculous, viral, fungal, parasitic infections, etc. Early etiological treatment is often the most crucial means to reduce the mortality rate of patients with central nervous system infections, reduce complications and sequelae, and improve prognosis. The initial clinical ...read more
Techniques of Drug Repurposing: Delivering a new life to Herbs & Drugs
Of late, with the adaptation of innovative approaches and integration of advancements made towards medical sciences as well as the availability of a wide range of tools; several therapeutic challenges are being translated into viable clinical solutions, with a high degree of efficacy, safety, and selectivity. With a better understanding ...read more
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
-
Oxidative/Nitrosative Stress and Immuno-inflammatory Pathways in Depression: Treatment Implications
Current Pharmaceutical Design Involvement of the Endocannabinoid System in Motor Disorders
Current Medicinal Chemistry - Central Nervous System Agents Advances in Quantitative Mass Spectrometry Analysis: Weighing in on Isotope Coding and Label-Free Approaches for Expression and Functional Proteomics
Current Analytical Chemistry Neural Stem Cells and Human Induced Pluripotent Stem Cells to Model Rare CNS Diseases
CNS & Neurological Disorders - Drug Targets Nitric Oxide, Peroxynitrite, Peroxynitrous Acid, Nitroxyl, Nitrogen Dioxide, Nitrous Oxide: Biochemical Mechanisms and Bioaction
Current Bioactive Compounds The Dawn of Mitophagy: What Do We Know by Now?
Current Neuropharmacology Editorial (Thematic Issue: Molecular and Cellular Engineering Approaches for Neurological Disorders, Diseases, and Injuries Involved the Central and Peripheral Nervous Systems)
CNS & Neurological Disorders - Drug Targets Quantitative Molecular Imaging of Neuronal Nicotinic Acetylcholine Receptors in the Human Brain with A-85380 Radiotracers
Current Medical Imaging Applications of High Content Screening in Life Science Research
Combinatorial Chemistry & High Throughput Screening Does a Pro-Inflammatory Process Precede Alzheimers Disease and Mild Cognitive Impairment?
Current Alzheimer Research Skeletal Muscle in Motor Neuron Diseases: Therapeutic Target and Delivery Route for Potential Treatments
Current Drug Targets The Immune Protective Effect of the Mediterranean Diet against Chronic Low-grade Inflammatory Diseases
Endocrine, Metabolic & Immune Disorders - Drug Targets Neuroprotective Effects of Low-dose Lithium in Individuals at Ultra-high Risk for Psychosis. A Longitudinal MRI/MRS Study
Current Pharmaceutical Design Therapy for Dominant Inherited Diseases by Allele-Specific RNA Interference: Successes and Pitfalls
Current Gene Therapy The Proteasome in Health and Disease
Current Pharmaceutical Design Decoding the Inter-Relationship between Sleep Disorders and Alzheimer’s Disease Pathogenesis
CNS & Neurological Disorders - Drug Targets Novel Therapeutic Targets for the Treatment of Depression
Current Medicinal Chemistry - Central Nervous System Agents Mitochondria as an Easy Target to Oxidative Stress Events in Parkinson's Disease
CNS & Neurological Disorders - Drug Targets p75NTR as a Therapeutic Target for Neuropsychiatric Diseases
Current Molecular Pharmacology Polymerase Delta in Eukaryotes: How is It Transiently Exchanged with Specialized DNA Polymerases During Translesion DNA Synthesis?
Current Protein & Peptide Science