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Article

A Point Mutation in Translation Initiation Factor 2B Leads to a Continuous Hyper Stress State in Oligodendroglial-Derived Cells

21 Nov 2008

Abstract excerpt

BACKGROUND: Mutations in eukaryotic translation initiation factor 2B (eIF2B) cause Childhood Ataxia with CNS Hypomyelination (CACH), also known as Vanishing White Matter disease (VWM). The disease is manifested by loss of brain myelin upon physiological stress. In a previous study, we showed that fibroblasts isolated from CACH/VWM patients are hypersensitive to pharmacologically-induced endoplasmic reticulum (ER)...

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PMID: 19023445
DOI: 10.1371/journal.pone.0003783

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A Point Mutation in Translation Initiation Factor 2B Leads to a Continuous Hyper Stress State in Oligodendroglial-Derived CellsPMID 19023445Read ↗

Heightened stress response in primary fibroblasts expressing mutant eIF2B genes from CACH/VWM leukodystrophy patients.Semantic proximity Read ↗

Adult mouse eIF2Bε Arg191His astrocytes display a normal integrated stress response in vitro.Semantic proximity Read ↗

EIF2B2 mutations in vanishing white matter disease hypersuppress translation and delay recovery during the integrated stress response.Semantic proximity Read ↗

eIF2B activator prevents neurological defects caused by a chronic Integrated Stress ResponseSemantic proximity Read ↗

Vanishing white matter disease expression of truncated EIF2B5 activates induced stress response.Semantic proximity Read ↗

Vanishing White Matter Disease Expression of Truncated EIF2B5 Activates Induced Stress ResponseSemantic proximity Read ↗

eIF2B localisation and its regulation during the integrated stress response is cell type specificSemantic proximity Read ↗

Mutations causing childhood ataxia with central nervous system hypomyelination reduce eukaryotic initiation factor 2B complex formation and activity.Semantic proximity Read ↗

Different Eukaryotic Initiation Factor 2Bε Mutations Lead to Various Degrees of Intolerance to the Stress of Endoplasmic Reticulum in Oligodendrocytes.Semantic proximity Read ↗

Paradoxical Sensitivity to an Integrated Stress Response Blocking Mutation in Vanishing White Matter Cells.Semantic proximity Read ↗

eIF2B Activator Rescues Neonatal Lethality of an eIF2Bα Sugar Phosphate Binding Mutation Associated with Vanishing White Matter DiseaseSemantic proximity Read ↗

Integrated Stress Response Deregulation underlies Vanishing White Matter and is a target for therapySemantic proximity Read ↗

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