Abstract
During the last thirty years, part of the scientific community focused on the mechanisms by which a naturally occurring protein called cellular prion (PrPc) converts into a protease-resistant isoform (PrPsc) responsible for fatal Transmissible Spongiform Encephalopathies (TSE). Concomitantly, the physiology of PrPc has also been studied. PrPc undergoes proteolytic attacks leading to both membrane-attached and secreted fragments, the nature of which differs in normal and TSE-affected human brains. Does proteolysis of PrPc correspond to an inactivating mechanism impairing the biological function of the protein, or alternatively, does it represent a maturation process allowing the produced fragments to trigger their own physiological function? Here we review the mechanisms involved in the production of PrPc catabolites and we focus on the function of PrPc and its derived fragments in the cell death/ survival regulation in the nervous system.
Keywords: ADAM, α-secretase-derived fragments, apoptosis, βAPP, cellular Prion protein, neuroprotection, p53, PRNP gene, infectious agent, Creutzfeld-Jacob Disease, synthetic peptide, proteolysis, neurodegeneration, brain pathologies, amyloid plaques
Current Molecular Medicine
Title: Cellular Prion and its Catabolites in the Brain: Production and Function
Volume: 12 Issue: 3
Author(s): M.-V. Guillot-Sestier and F. Checler
Affiliation:
Keywords: ADAM, α-secretase-derived fragments, apoptosis, βAPP, cellular Prion protein, neuroprotection, p53, PRNP gene, infectious agent, Creutzfeld-Jacob Disease, synthetic peptide, proteolysis, neurodegeneration, brain pathologies, amyloid plaques
Abstract: During the last thirty years, part of the scientific community focused on the mechanisms by which a naturally occurring protein called cellular prion (PrPc) converts into a protease-resistant isoform (PrPsc) responsible for fatal Transmissible Spongiform Encephalopathies (TSE). Concomitantly, the physiology of PrPc has also been studied. PrPc undergoes proteolytic attacks leading to both membrane-attached and secreted fragments, the nature of which differs in normal and TSE-affected human brains. Does proteolysis of PrPc correspond to an inactivating mechanism impairing the biological function of the protein, or alternatively, does it represent a maturation process allowing the produced fragments to trigger their own physiological function? Here we review the mechanisms involved in the production of PrPc catabolites and we focus on the function of PrPc and its derived fragments in the cell death/ survival regulation in the nervous system.
Export Options
About this article
Cite this article as:
Guillot-Sestier M.-V. and Checler F., Cellular Prion and its Catabolites in the Brain: Production and Function, Current Molecular Medicine 2012; 12 (3) . https://dx.doi.org/10.2174/156652412799218912
DOI https://dx.doi.org/10.2174/156652412799218912 |
Print ISSN 1566-5240 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5666 |
- 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
-
The Therapeutic use of the Zonulin Inhibitor AT-1001 (Larazotide) for a Variety of Acute and Chronic Inflammatory Diseases
Current Medicinal Chemistry Necessity for Re-Vascularization after Spinal Cord Injury and the Search for Potential Therapeutic Options
Current Neurovascular Research TiO2-Nanowired Delivery of Mesenchymal Stem Cells Thwarts Diabetes- Induced Exacerbation of Brain Pathology in Heat Stroke: An Experimental Study in the Rat Using Morphological and Biochemical Approaches
CNS & Neurological Disorders - Drug Targets Identifying S100B as a Biomarker and a Therapeutic Target For Brain Injury and Multiple Diseases
Current Medicinal Chemistry CD36 as a Multiple-Ligand Signaling Receptor in Atherothrombosis
Cardiovascular & Hematological Agents in Medicinal Chemistry Synthesis and Biological Activity of Chiral Dihydropyrazole: Potential Lead for Drug Design
Mini-Reviews in Medicinal Chemistry Stem Cells as a Novel Tool for Drug Screening and Treatment of Degenerative Diseases
Current Pharmaceutical Design Modulation of Cellular Function by TAT Mediated Transduction of Full Length Proteins
Current Protein & Peptide Science Pregnancy and Sleep Apnea
Current Respiratory Medicine Reviews Importance of Aquaporins in the Physiopathology of Brain Edema
Current Pharmaceutical Design Dynamic Factors Controlling Targeting Nanocarriers to Vascular Endothelium
Current Drug Metabolism Protein Transduction Revisited: Novel Insights Into the Mechanism Underlying Intracellular Delivery of Proteins
Current Pharmaceutical Design Sesbania: A Prospective Candidate to be Excavated for Anticancer Drugs
The Natural Products Journal Apelin/Apelin Receptor System: Molecular Characteristics, Physiological Roles, and Prospects as a Target for Disease Prevention and Pharmacotherapy
Current Molecular Pharmacology Could Better Phenotyping Small Vessel Disease Provide New Insights into Alzheimer Disease and Improve Clinical Trial Outcomes?
Current Alzheimer Research The Variable Presentations of Glycogen Storage Disease Type IV: A Review of Clinical, Enzymatic and Molecular Studies
Current Molecular Medicine Pigment Epithelium-derived Factor (PEDF) and Cardiometabolic Disorders
Current Pharmaceutical Design Clinical Trials with Intracerebral Convection-Enhanced Delivery of Targeted Toxins in Malignant Glioma
Reviews on Recent Clinical Trials The Urokinase Receptor System, A Key Regulator at the Intersection between Inflammation, Immunity, and Coagulation
Current Pharmaceutical Design Search and Rescue: Identification of Cannabinoid Actions Relevant for Neuronal Survival and Protection
Current Neuropharmacology