Stoppini, M;
Bellotti, V;
(2015)
Systemic amyloidosis: lessons from β2-microglobulin.
Journal of Biological Chemistry
, 290
(16)
pp. 9951-9958.
10.1074/jbc.R115.639799.
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Abstract
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.
| Type: | Article |
|---|---|
| Title: | Systemic amyloidosis: lessons from β2-microglobulin. |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1074/jbc.R115.639799 |
| Publisher version: | http://dx.doi.org/10.1074/jbc.R115.639799 |
| Additional information: | © 2015 by The American Society for Biochemistry and Molecular Biology, Inc. Author's Choice—Final version full access. Creative Commons Attribution Unported License applies to Author Choice Articles |
| Keywords: | Amyloid, Atomic Force Microscopy (AFM), Fibrillogenesis in Vitro, Genetic Variant Asp76Asn, Mechanism of Amyloidogenesis, Protein Aggregation, Protein Misfolding, Protein Structure, β2-Microglobulin, Amyloid, Amyloidosis, Doxycycline, Humans, Models, Molecular, Mutation, Protein Aggregation, Pathological, Protein Conformation, Renal Dialysis, Single-Chain Antibodies, beta 2-Microglobulin |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1472259 |
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