Abstract
The PrP propensity to adopt different structures is tightly linked to transmissible spongiform encephalopathies (TSE) which include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scjeinker (GSS) and Kuru syndrome. In most cases, TSE is associated with the accumulation in the brain of an abnormally folded protease-resistant protein, PrPSc or PrPres, which is derived from a cellular host-encoded protease-sensitive conformer, designated PrPC. The prion propagation in the brain is postulated to occur via a conformational change of PrPC into the amyloidogenic form PrPSc, characterized by a high β sheet content. The characterization of PrPSC oligomers as well as their biological activity is currently an area of active research. Indeed, PrPSc structural diversity was proposed several years ago as a hypothesis to explain the origin of “prion strain” diversity. As prion pathologies belong to protein miss-assembly diseases, investigation of PrP conformational dynamics and, more precisely, oligomerization pathways exploration will help to acheave a better understanding of the pathological events at the molecular level.
Keywords: transmissible spongiform encephalopathies, kinetic intermediate, oligomerization, soluble oligomers, protein misassembly, amyloid, catalyse, nucleus, seed
Current Alzheimer Research
Title: Prion Protein Oligomerization
Volume: 5 Issue: 6
Author(s): H. Rezaei
Affiliation:
Keywords: transmissible spongiform encephalopathies, kinetic intermediate, oligomerization, soluble oligomers, protein misassembly, amyloid, catalyse, nucleus, seed
Abstract: The PrP propensity to adopt different structures is tightly linked to transmissible spongiform encephalopathies (TSE) which include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scjeinker (GSS) and Kuru syndrome. In most cases, TSE is associated with the accumulation in the brain of an abnormally folded protease-resistant protein, PrPSc or PrPres, which is derived from a cellular host-encoded protease-sensitive conformer, designated PrPC. The prion propagation in the brain is postulated to occur via a conformational change of PrPC into the amyloidogenic form PrPSc, characterized by a high β sheet content. The characterization of PrPSC oligomers as well as their biological activity is currently an area of active research. Indeed, PrPSc structural diversity was proposed several years ago as a hypothesis to explain the origin of “prion strain” diversity. As prion pathologies belong to protein miss-assembly diseases, investigation of PrP conformational dynamics and, more precisely, oligomerization pathways exploration will help to acheave a better understanding of the pathological events at the molecular level.
Export Options
About this article
Cite this article as:
Rezaei H., Prion Protein Oligomerization, Current Alzheimer Research 2008; 5 (6) . https://dx.doi.org/10.2174/156720508786898497
DOI https://dx.doi.org/10.2174/156720508786898497 |
Print ISSN 1567-2050 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5828 |
Call for Papers in Thematic Issues
New Advances in the Prevention, Diagnosis, Treatment, and Rehabilitation of Alzheimer's Disease
Aims and Scope: Introduction: Alzheimer's disease (AD) poses a significant global health challenge, with an increasing prevalence that demands concerted efforts to advance our understanding and strategies for prevention, diagnosis, treatment, and rehabilitation. This thematic issue aims to bring together cutting-edge research and innovative approaches from multidisciplinary perspectives to address ...read more
Current updates on the Role of Neuroinflammation in Neurodegenerative Disorders
Neuroinflammation is an invariable hallmark of chronic and acute neurodegenerative disorders and has long been considered a potential drug target for Alzheimer?s disease (AD) and dementia. Significant evidence of inflammatory processes as a feature of AD is provided by the presence of inflammatory markers in plasma, CSF and postmortem brain ...read more
Deep Learning for Advancing Alzheimer's Disease Research
Alzheimer's disease (AD) poses a significant global health challenge, with an increasing number of individuals affected yearly. Deep learning, a subfield of artificial intelligence, has shown immense potential in various domains, including healthcare. This thematic issue of Current Alzheimer Research explores the application of deep learning techniques in advancing our ...read more
Diagnostic and therapeutic biomarkers of dementia
Dementia affects 18 million people worldwide. Dementia is a syndrome of symptoms caused by brain disease, usually chronic or progressive, clinically characterized by multiple impairments of higher cortical functions such as memory, thinking, orientation, and learning. In addition, in the course of dementia, cognitive deficits are observed, which often hinder ...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
-
Astatine Radiopharmaceuticals: Prospects and Problems
Current Radiopharmaceuticals Epigenetics in Medullary Thyroid Cancer: From Pathogenesis to Targeted Therapy
Recent Patents on Anti-Cancer Drug Discovery Peroxisome Proliferator Activated Receptor Gamma Coactivator 1 Alpha: An Emerging Target for Neuroprotection in Parkinson’s Disease
CNS & Neurological Disorders - Drug Targets Polyamine Modulation of NMDARs as a Mechanism to Reduce Effects of Alcohol Dependence
Recent Patents on CNS Drug Discovery (Discontinued) Vaccination and Antiviral Treatment of Neglected Diseases Caused by Flaviviral Infections
Current Medicinal Chemistry Activation of Brain Endothelium by Soluble Aggregates of the Amyloid-β Protein Involves Nuclear Factor-κB
Current Alzheimer Research Recent Achievement in the Synthesis of Benzo[b]furans
Current Organic Synthesis Formulation and Characterization of Rutin Loaded Chitosan-alginate Nanoparticles: Antidiabetic and Cytotoxicity Studies
Current Drug Delivery mTOR Targeted Cancer Chemoprevention by Flavonoids
Current Medicinal Chemistry Lipid Modulation of Intravascular and Cellular Sodium Handling:Mechanistic Insights and Potential Clinical Implications
Current Vascular Pharmacology The Impact of Proteomics in the Understanding of the Molecular Basis of Paclitaxel-Resistance in Ovarian Tumors
Current Cancer Drug Targets Plant Polyphenols and Tumors: From Mechanisms to Therapies, Prevention, and Protection Against Toxicity of Anti-Cancer Treatments
Current Medicinal Chemistry A Survey of Peptides with Effective Therapeutic Potential in Alzheimer’s Disease Rodent Models or in Human Clinical Studies
Mini-Reviews in Medicinal Chemistry Specific Targeting of Akt Kinase Isoforms: Taking the Precise Path for Prevention and Treatment of Cancer
Current Drug Targets Nitroxides as Cancer Imaging Agents
Anti-Cancer Agents in Medicinal Chemistry Survival Signaling Pathways Activated by NMDA Receptors
Current Topics in Medicinal Chemistry Conformational Changes of Prion Protein and Nucleic Acid Arising from their Interaction and Relation of the Altered Structures in Causing Prion Disease
Mini-Reviews in Medicinal Chemistry Establishing Genomic/Transcriptomic Links Between Alzheimer’s Disease and Type 2 Diabetes Mellitus by Meta-Analysis Approach
CNS & Neurological Disorders - Drug Targets Hypoxia-Inducible Factor-1 as Regulator of Angiogenesis in Rheumatoid Arthritis - Therapeutic Implications
Current Medicinal Chemistry Hyperglycemia, Hypoglycemia and Dementia: Role of Mitochondria and Uncoupling Proteins
Current Molecular Medicine