The unfolded protein response in vanishing white matter disease.
about
Developmental splicing deregulation in leukodystrophies related to EIF2B mutationsEvaluation of the endoplasmic reticulum-stress response in eIF2B-mutated lymphocytes and lymphoblasts from CACH/VWM patientsAutophagy promotes oligodendrocyte survival and function following dysmyelination in a long-lived myelin mutantDefective glial maturation in vanishing white matter disease.A point mutation in translation initiation factor eIF2B leads to function--and time-specific changes in brain gene expression.Sensitivity and specificity of decreased CSF asialotransferrin for eIF2B-related disorder.Impaired eukaryotic translation initiation factor 2B activity specifically in oligodendrocytes reproduces the pathology of vanishing white matter disease in mice.Endoplasmic reticulum stress in disorders of myelinating cells.Impaired eIF2B activity in ligodendrocytes contributes to VWMD pathogenesis.Biochemical effects of mutations in the gene encoding the alpha subunit of eukaryotic initiation factor (eIF) 2B associated with Vanishing White Matter diseaseMutation of sec63 in zebrafish causes defects in myelinated axons and liver pathologyDifferent Eukaryotic Initiation Factor 2Bε Mutations Lead to Various Degrees of Intolerance to the Stress of Endoplasmic Reticulum in OligodendrocytesA point mutation in translation initiation factor 2B leads to a continuous hyper stress state in oligodendroglial-derived cells.The unfolded protein response is activated in pretangle neurons in Alzheimer's disease hippocampusGeneral control nonderepressible 2 (GCN2) kinase protects oligodendrocytes and white matter during branched-chain amino acid deficiency in miceMyelin under stressASTROCYTES: EMERGING STARS IN LEUKODYSTROPHY PATHOGENESIS.Endoplasmic reticulum quality control and dysmyelination.Translational control by eIF2α in neurons: Beyond the stress response.Endoplasmic reticulum stress and the unfolded protein response in disorders of myelinating gliaProtein kinase R(PKR)-like endoplasmic reticulum kinase (PERK) inhibitors: a patent review (2010-2015).Genetic Leukoencephalopathies in Adults.Mutant eIF2B leads to impaired mitochondrial oxidative phosphorylation in vanishing white matter disease.Endoplasmic Reticulum Protein Quality Control Failure in Myelin Disorders.Fine-tuning PERK signaling for neuroprotection.EIF2S3 Mutations Associated with Severe X-Linked Intellectual Disability Syndrome MEHMO.Cannabidiol protects oligodendrocyte progenitor cells from inflammation-induced apoptosis by attenuating endoplasmic reticulum stress.Early reduction of total N-acetyl-aspartate-compounds in patients with classical vanishing white matter disease. A long-term follow-up MRS study.Regulation of mRNA Translation in Neurons-A Matter of Life and Death.Axonal abnormalities in vanishing white matter.EIF2B2 mutations in Vanishing White Matter Disease hyper- suppress translation and delay recovery during the integrated stress response.Adult mouse eIF2Bε Arg191His astrocytes display a normal integrated stress response in vitro.The small molecule ISRIB rescues the stability and activity of Vanishing White Matter Disease eIF2B mutant complexes.Genetic and Clinical Heterogeneity in eIF2B-Related DisorderUpdate on leukodystrophies
P2860
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P2860
The unfolded protein response in vanishing white matter disease.
description
2005 nî lūn-bûn
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2005年の論文
@ja
2005年学术文章
@wuu
2005年学术文章
@zh-cn
2005年学术文章
@zh-hans
2005年学术文章
@zh-my
2005年学术文章
@zh-sg
2005年學術文章
@yue
2005年學術文章
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2005年學術文章
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name
The unfolded protein response in vanishing white matter disease.
@en
The unfolded protein response in vanishing white matter disease.
@nl
type
label
The unfolded protein response in vanishing white matter disease.
@en
The unfolded protein response in vanishing white matter disease.
@nl
prefLabel
The unfolded protein response in vanishing white matter disease.
@en
The unfolded protein response in vanishing white matter disease.
@nl
P2093
P2860
P1476
The unfolded protein response in vanishing white matter disease
@en
P2093
Barbara van Kollenburg
Gert C Scheper
Gesina Bertrand
J Patrick van der Voorn
James M Powers
Marjo S van der Knaap
P2860
P304
P356
10.1097/01.JNEN.0000178446.41595.3A
P577
2005-09-01T00:00:00Z