UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Systemic amyloidosis: lessons from β2-microglobulin.

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. Green open access

[thumbnail of Article]
Preview
Text (Article)
J. Biol. Chem.-2015-Stoppini-9951-8.pdf

Download (2MB) | Preview

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
Downloads since deposit
117Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item