Mechanisms and Functions of Spatial Protein Quality Control.
Annu Rev Biochem
; 86: 97-122, 2017 06 20.
Article
em En
| MEDLINE
| ID: mdl-28489421
ABSTRACT
A healthy proteome is essential for cell survival. Protein misfolding is linked to a rapidly expanding list of human diseases, ranging from neurodegenerative diseases to aging and cancer. Many of these diseases are characterized by the accumulation of misfolded proteins in intra- and extracellular inclusions, such as amyloid plaques. The clear link between protein misfolding and disease highlights the need to better understand the elaborate machinery that manages proteome homeostasis, or proteostasis, in the cell. Proteostasis depends on a network of molecular chaperones and clearance pathways involved in the recognition, refolding, and/or clearance of aberrant proteins. Recent studies reveal that an integral part of the cellular management of misfolded proteins is their spatial sequestration into several defined compartments. Here, we review the properties, function, and formation of these compartments. Spatial sequestration plays a central role in protein quality control and cellular fitness and represents a critical link to the pathogenesis of protein aggregation-linked diseases.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Envelhecimento
/
Chaperonas Moleculares
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Doenças Neurodegenerativas
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Deficiências na Proteostase
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Agregação Patológica de Proteínas
Limite:
Humans
Idioma:
En
Revista:
Annu Rev Biochem
Ano de publicação:
2017
Tipo de documento:
Article