Systemic amyloidoses.
Annu Rev Biochem
; 82: 745-74, 2013.
Article
em En
| MEDLINE
| ID: mdl-23451869
ABSTRACT
The amyloidoses are a group of protein misfolding diseases in which the precursor protein undergoes a conformational change that triggers the formation of amyloid fibrils in different tissues and organs, causing cell death and organ failure. Amyloidoses can be either localized or systemic. In localized amyloidosis, amyloid deposits form at the site of precursor protein synthesis, whereas in systemic amyloidosis, amyloid deposition occurs distant from the site of precursor protein secretion. We review the type of proteins and cells involved and what is known about the complex pathophysiology of these diseases. We focus on light chain amyloidosis to illustrate how biochemical and biophysical studies have led to a deeper understanding of the pathogenesis of this devastating disease. We also review current cellular, tissue, and animal models and discuss the challenges and opportunities for future studies of the systemic amyloidoses.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Precursores de Proteínas
/
Cadeias Leves de Imunoglobulina
/
Dobramento de Proteína
/
Amiloide
/
Amiloidose
Tipo de estudo:
Etiology_studies
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Annu Rev Biochem
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Estados Unidos