Protein Aggregation
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Giampaolo Merlini
Abstract
Protein aggregation occurs in vivo as a result of improper folding or misfolding. Diverse diseases arise from protein misfolding and are now grouped under the term “protein conformational diseases”, including most of the neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, the prion encephalopathies and Huntington's disease, as well as cystic fibrosis, sickle cell anemia and other less common conditions. The hallmark event in these diseases is a change in the secondary and/or tertiary structure of a normal, functional protein, leading to the formation of protein aggregates with various supramolecular organizations. In most cases the aggregates are organized in structurally well-defined fibrils forming amyloid deposits. The crucial feature of the amyloidogenic proteins is their structural instability induced either by mutations, post-translational modifications, or local conditions, such as pH, temperature, and co-solutes. The conformational change may promote the disease either by gain of a toxic activity or by the lack of biological function of the natively folded protein. As different molecular mechanisms are involved in the formation of the various forms of protein aggregates, the laboratory diagnostic approach remains frequently elusive.
Copyright © 2001 by Walter de Gruyter GmbH & Co. KG
Articles in the same Issue
- The Present State of Protein Analysis and Interpretation
- In Memoriam Carl-Bertil Laurell
- From Paper Electrophoresis to Computer-supported Interpretation of Capillary Electrophoresis – Clinical Plasma Protein Analysis in Malmö, Sweden
- The Pavia Approach to Clinical Protein Analysis
- How the Foundation for Blood Research (FBR) Has Managed Serum Protein Testing for New England Clinicians
- Evidence-Based Laboratory Interpretation System Built on a Large Collection of Case Records with Well-Defined Diagnoses
- A Knowledge-Based System to Aid with the Clinical Interpretation of Complex Serum Protein Data
- Markers of the Acute Phase Response in Cardiovascular Disease: An Update
- Protein Aggregation
- Protein Standardization I: Protein Purification. Procedure for the Purification of Human Prealbumin, Orosomucoid and Transferrin as Primary Protein Preparations
- Protein Standardization II: Dry Mass Determination. Procedure for the Determination of the Dry Mass of a Pure Protein Preparation
- Protein Standardization III: Method Optimization. Basic Principles for Quantitative Determination of Human Serum Proteins on Automated Instruments Based on Turbidimetry or Nephelometry
- Protein Standardization IV: Value Transfer. Procedure for the Assignment of Serum Protein Values from a Reference Preparation to a Target Material
- Effect of Certified Reference Material 470 (CRM 470) on National Quality Assurance Programs for Serum Proteins in Europe
- Commutability of Serum Protein Values: Persisting Bias among Manufacturers Using Values Assigned from the Certified Reference Material 470 (CRM 470) in the United States
- The Existing Interim Consensus Reference Ranges and the Future Approach
- Using Multiples of the Median to Normalize Serum Protein Measurements
- Strategy for Determining Racial and Environmental Similarities and Differences for Plasma Proteins
- Standardization of Immunoassay for CRM-Related Proteins in Japan: From Evaluating CRM 470 to Setting Reference Intervals
- Soluble Transferrin Receptor (sTfR): Biological Variations and Reference Limits
- High Sensitivity C-Reactive Protein (CRP) Reference Intervals in the Elderly
- High Sensitivity Immunoassays for C-Reactive Protein: Promises and Pitfalls
- Meetings and Awards
Articles in the same Issue
- The Present State of Protein Analysis and Interpretation
- In Memoriam Carl-Bertil Laurell
- From Paper Electrophoresis to Computer-supported Interpretation of Capillary Electrophoresis – Clinical Plasma Protein Analysis in Malmö, Sweden
- The Pavia Approach to Clinical Protein Analysis
- How the Foundation for Blood Research (FBR) Has Managed Serum Protein Testing for New England Clinicians
- Evidence-Based Laboratory Interpretation System Built on a Large Collection of Case Records with Well-Defined Diagnoses
- A Knowledge-Based System to Aid with the Clinical Interpretation of Complex Serum Protein Data
- Markers of the Acute Phase Response in Cardiovascular Disease: An Update
- Protein Aggregation
- Protein Standardization I: Protein Purification. Procedure for the Purification of Human Prealbumin, Orosomucoid and Transferrin as Primary Protein Preparations
- Protein Standardization II: Dry Mass Determination. Procedure for the Determination of the Dry Mass of a Pure Protein Preparation
- Protein Standardization III: Method Optimization. Basic Principles for Quantitative Determination of Human Serum Proteins on Automated Instruments Based on Turbidimetry or Nephelometry
- Protein Standardization IV: Value Transfer. Procedure for the Assignment of Serum Protein Values from a Reference Preparation to a Target Material
- Effect of Certified Reference Material 470 (CRM 470) on National Quality Assurance Programs for Serum Proteins in Europe
- Commutability of Serum Protein Values: Persisting Bias among Manufacturers Using Values Assigned from the Certified Reference Material 470 (CRM 470) in the United States
- The Existing Interim Consensus Reference Ranges and the Future Approach
- Using Multiples of the Median to Normalize Serum Protein Measurements
- Strategy for Determining Racial and Environmental Similarities and Differences for Plasma Proteins
- Standardization of Immunoassay for CRM-Related Proteins in Japan: From Evaluating CRM 470 to Setting Reference Intervals
- Soluble Transferrin Receptor (sTfR): Biological Variations and Reference Limits
- High Sensitivity C-Reactive Protein (CRP) Reference Intervals in the Elderly
- High Sensitivity Immunoassays for C-Reactive Protein: Promises and Pitfalls
- Meetings and Awards
