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Article

The unfolded protein response in vanishing white matter disease.

Journal of neuropathology and experimental neurology - 1 Sept 2005

van der Voorn J Patrick, van Kollenburg Barbara, Bertrand Gesina, Van Haren Keith, Scheper Gert C, Powers James M, van der Knaap Marjo S

Abstract excerpt

Leukoencephalopathy with vanishing white matter (VWM) is an autosomal-recessive disorder in which febrile infections may provoke major neurologic deterioration. Characteristic pathologic findings include cystic white matter degeneration, foamy oligodendrocytes, dysmorphic astrocytes and oligodendrocytes, oligodendrocytosis, and apoptotic losses of oligodendrocytes. VWM is caused by mutations in eukaryotic...

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Source

Identifiers

PMID: 16141786
DOI: 10.1097/01.jnen.0000178446.41595.3a

Genes

ATF4
EBP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Adult-onset leukoencephalopathy with vanishing white matter with compound heterozygous EIF2B3 gene variantsSemantic proximity Read ↗

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The Translational Landscape of Reactive Astrocytes Reveals the Impact of eIF2B-mediated Dysregulation in VWM DiseaseSemantic proximity Read ↗

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Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter.Semantic proximity Read ↗

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