Article
Mutational analysis of the alpha subunit of eIF2B provides insights into the role of eIF2B bodies in translational control and VWM disease.
The Journal of biological chemistry - 1 Jan 2000
Norris Karl, Hodgson Rachel E, Dornelles Tawni, Allen K Elizabeth, Abell Ben M, Ashe Mark P, Campbell Susan G
Abstract excerpt
Eukaryotic initiation factor 2B (eIF2B) serves as a vital control point within protein synthesis and regulates translation initiation in response to cellular stress. Mutations within eIF2B result in the fatal disease, leukoencephalopathy with vanishing white matter (VWM). Previous biochemical studies on VWM mutations have illustrated that changes in the activity of eIF2B poorly correlate with disease severity....
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