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Article

Vanishing white matter: deregulated integrated stress response as therapy target

18 Jul 2019

Abstract excerpt

OBJECTIVE: Vanishing white matter (VWM) is a fatal, stress-sensitive leukodystrophy that mainly affects children and is currently without treatment. VWM is caused by recessive mutations in eukaryotic initiation factor 2B (eIF2B) that is crucial for initiation of mRNA translation and its regulation during the integrated stress response (ISR). Mutations reduce eIF2B activity. VWM pathomechanisms remain unclear. In...

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PMID: 31402619
DOI: 10.1002/acn3.50826

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