Systemic amyloidosis: lessons from ß2-microglobulin.
J Biol Chem
; 290(16): 9951-8, 2015 Apr 17.
Article
em En
| MEDLINE
| ID: mdl-25750126
ABSTRACT
ß2-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 ß2-microglobulin mechanism has been scrutinized in depth for the last three decades, the comparative analysis of molecular and pathological properties of wild type ß2-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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Microglobulina beta-2
/
Agregação Patológica de Proteínas
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Amiloide
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Amiloidose
Tipo de estudo:
Etiology_studies
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Prognostic_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article