Distribution of Cellular Prion Protein in Normal Human Cerebral Cortex – Does It Have Relevance to Creutzfeldt-Jakob Disease?
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Gerard H. Jansen
Abstract
Creutzfeldt-Jakob disease and bovine spongiform encephalopathy are the best known forms of prion diseases. A basis for their pathogenesis is the transformation of normal prion protein to abnormal prion protein. This would mean that either loss of normal function or a gain of a toxic function of the prion protein would play a major role. Since the prime target for Creutzfeldt-Jakob disease in humans is the neocortex, and the intracort ical distribution of the destructive process in prion diseases appears not to be haphazard, it may be that a clear cortical study of normal prion protein production in the premorbid human neocortex might contribute to insight in the pathogenesis of prion diseases. As no such study is available, we performed a detailed study in normal human cortex using immunohistochemistry for prion protein, in both frozen and vibratomised tissue, and in situ hybridisation for prion protein mRNA. We have found normal prion protein production mainly in the upper cortical neurons in neocortex and Purkinje cells in the cerebellum. This finding implicates that normal prion protein is more important as an anti-apoptotic signal in disease than abnormal prion protein is as a toxic substance.
Copyright © 2001 by Walter de Gruyter GmbH & Co. KG
Articles in the same Issue
- IFCC/ Beckman Coulter Inc. Conference Frontiers in Molecular Basis of Diseases: Cell Biology of Neuronal Dysfunction, Paris, October 12-13, 2000
- Distribution of Cellular Prion Protein in Normal Human Cerebral Cortex – Does It Have Relevance to Creutzfeldt-Jakob Disease?
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- Aggregation-Dependent Interaction of the Alzheimers β-Amyloid and Microglia
- β-Amyloid-Induced Cytotoxicity, Peroxide Generation and Blockade of Glutamate Uptake in Cultured Astrocytes
- Protein S-100B: A Serum Marker for Ischemic and Infectious Injury of Cerebral Tissue
- Reporting Cerebrospinal Fluid Data: Knowledge Base and Interpretation Software
- The Intrathecal Humoral Immune Response: Laboratory Analysis and Clinical Relevance
- Source of Endothelin-1 in Subarachnoid Hemorraghe
- Polymorphism of Apoprotein E (APOE), Methylenetetrahydrofolate Reductase (MTHFR) and Paraoxonase (PON1) Genes in Patients with Cerebrovascular Disease
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Articles in the same Issue
- IFCC/ Beckman Coulter Inc. Conference Frontiers in Molecular Basis of Diseases: Cell Biology of Neuronal Dysfunction, Paris, October 12-13, 2000
- Distribution of Cellular Prion Protein in Normal Human Cerebral Cortex – Does It Have Relevance to Creutzfeldt-Jakob Disease?
- Caspase-3 Apoptotic Signaling Following Injury to the Central Nervous System
- Parkinsons Disease and other α-Synucleinopathies
- Aggregation-Dependent Interaction of the Alzheimers β-Amyloid and Microglia
- β-Amyloid-Induced Cytotoxicity, Peroxide Generation and Blockade of Glutamate Uptake in Cultured Astrocytes
- Protein S-100B: A Serum Marker for Ischemic and Infectious Injury of Cerebral Tissue
- Reporting Cerebrospinal Fluid Data: Knowledge Base and Interpretation Software
- The Intrathecal Humoral Immune Response: Laboratory Analysis and Clinical Relevance
- Source of Endothelin-1 in Subarachnoid Hemorraghe
- Polymorphism of Apoprotein E (APOE), Methylenetetrahydrofolate Reductase (MTHFR) and Paraoxonase (PON1) Genes in Patients with Cerebrovascular Disease
- Neurotrophic Factor Therapy – Prospects and Problems
- Cell Therapy and Transplantation in Parkinsons Disease
- Matrix Metalloproteinases: Potential Therapeutic Target in Spinal Cord Injury
