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The small molecule ISRIB rescues the stability and activity of Vanishing White Matter Disease eIF2B mutant complexes

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...

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Detalles Bibliográficos
Autores principales: Wong, Yao Liang, LeBon, Lauren, Edalji, Rohinton, Lim, Hock Ben, Sun, Chaohong, Sidrauski, Carmela
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5829914/
https://www.ncbi.nlm.nih.gov/pubmed/29489452
http://dx.doi.org/10.7554/eLife.32733