The small molecule ISRIB rescues the stability and activity of Vanishing White Matter Disease eIF2B mutant complexes.
Elife
; 72018 02 28.
Article
em En
| MEDLINE
| ID: mdl-29489452
ABSTRACT
eIF2B is a dedicated guanine nucleotide exchange factor for eIF2, the GTPase that is essential to initiate mRNA translation. The integrated stress response (ISR) signaling pathway inhibits eIF2B activity, attenuates global protein synthesis and upregulates a set of stress-response proteins. Partial loss-of-function mutations in eIF2B cause a neurodegenerative disorder called Vanishing White Matter Disease (VWMD). Previously, we showed that the small molecule ISRIB is a specific activator of eIF2B (Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Fármacos Neuroprotetores
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Fator de Iniciação 2B em Eucariotos
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Ativadores de Enzimas
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Cicloexilaminas
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Proteínas Mutantes
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Leucoencefalopatias
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Acetamidas
Limite:
Humans
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article