Systemic Amyloidosis: Lessons from β2-Microglobulin
Copyright policy )Systemic Amyloidosis: Lessons from β2-Microglobulin
β2-Microglobulin is responsible for systemic amyloidosis affecting patients undergoing long-term hemodialysis. Its genetic variant D76N causes a very rare form of familial systemic amyloidosis. These two types of amyloidoses differ significantly in terms of the tissue localization of deposits and for major pathological features. Considering how the amyloidogenesis of the β2-microglobulin mechanism has been scrutinized in depth for the last three decades, the comparative analysis of molecular and pathological properties of wild type β2-microglobulin and of the D76N variant offers a unique opportunity to critically reconsider the current understanding of the relation between the protein's structural properties and its pathologic behavior.
- UNIVERSITY COLLEGE LONDON, Bartlett School of Planning United Kingdom
- University College London United Kingdom
- University of Pavia Italy
Models, Molecular, Protein Structure, Amyloid, Protein Conformation, Mechanism of Amyloidogenesis, 610, Atomic Force Microscopy (AFM), Protein Aggregation, Pathological, Models, Pathological, Renal Dialysis, 616, Humans, Fibrillogenesis in Vitro, β2-Microglobulin, Molecular Mutation Protein Aggregation, Amyloid/antagonists & inhibitors/*chemistry/metabolism Amyloidosis/classification/*drug therapy/etiology/genetics Doxycycline/pharmacology Humans Models, Molecular, Minireviews, Amyloidosis, Protein Aggregation, Protein Misfolding, Doxycycline, Mutation, beta 2-Microglobulin, Genetic Variant Asp76Asn, Pathological/metabolism/*prevention & control Protein Conformation Renal Dialysis/adverse effects Single-Chain Antibodies/pharmacology beta 2-Microglobulin/antagonists & inhibitors/*chemistry/metabolism, Single-Chain Antibodies
Models, Molecular, Protein Structure, Amyloid, Protein Conformation, Mechanism of Amyloidogenesis, 610, Atomic Force Microscopy (AFM), Protein Aggregation, Pathological, Models, Pathological, Renal Dialysis, 616, Humans, Fibrillogenesis in Vitro, β2-Microglobulin, Molecular Mutation Protein Aggregation, Amyloid/antagonists & inhibitors/*chemistry/metabolism Amyloidosis/classification/*drug therapy/etiology/genetics Doxycycline/pharmacology Humans Models, Molecular, Minireviews, Amyloidosis, Protein Aggregation, Protein Misfolding, Doxycycline, Mutation, beta 2-Microglobulin, Genetic Variant Asp76Asn, Pathological/metabolism/*prevention & control Protein Conformation Renal Dialysis/adverse effects Single-Chain Antibodies/pharmacology beta 2-Microglobulin/antagonists & inhibitors/*chemistry/metabolism, Single-Chain Antibodies
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