Molecular and cellular aspects of protein misfolding and disease.
FASEB J
; 22(7): 2115-33, 2008 Jul.
Article
em En
| MEDLINE
| ID: mdl-18303094
ABSTRACT
Proteins are essential elements for life. They are building blocks of all organisms and the operators of cellular functions. Humans produce a repertoire of at least 30,000 different proteins, each with a different role. Each protein has its own unique sequence and shape (native conformation) to fulfill its specific function. The appearance of incorrectly shaped (misfolded) proteins occurs on exposure to environmental changes. Protein misfolding and the subsequent aggregation is associated with various, often highly debilitating, diseases for which no sufficient cure is available yet. In the first part of this review we summarize the structural composition of proteins and the current knowledge of underlying forces that lead proteins to lose their native structure. In the second and third parts we describe the molecular and cellular mechanisms that are associated with protein misfolding in disease. Finally, in the last part we portray recent efforts to develop treatments for protein misfolding diseases.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças Vasculares
/
Proteínas
/
Doença
/
Dobramento de Proteína
Limite:
Humans
Idioma:
En
Revista:
FASEB J
Ano de publicação:
2008
Tipo de documento:
Article