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Related Concept Videos

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Chemistry is the study of matter and the changes it undergoes. Matter is anything that has mass and occupies space. Matter is all around us; the air, water, soil, mountains, even our bodies are all examples of matter. Matter is divided into three states — solid, liquid, and gas — that are commonly found on earth. The fourth state of matter, plasma, occurs naturally in the interiors of stars. 
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Related Experiment Video

Updated: Feb 10, 2026

A Versatile Murine Model of Subcortical White Matter Stroke for the Study of Axonal Degeneration and White Matter Neurobiology
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Vanishing white matter: a leukodystrophy due to astrocytic dysfunction.

Marianna Bugiani1, Caroline Vuong1, Marjolein Breur1

  • 1Departments of Pathology, Child Neurology, and Functional Genomics, VU University Medical Center, Amsterdam Neuroscience, Amsterdam, The Netherlands.

Brain Pathology (Zurich, Switzerland)
|May 10, 2018
PubMed
Summary

Vanishing white matter disease (VWM) is a prevalent leukodystrophy caused by mutations in the eukaryotic translation initiation factor 2B (eIF2B) gene. This study explores VWM

Keywords:
astrocytesleukodystrophyvanishing white matter

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Vanishing white matter disease (VWM) is a prevalent, often fatal, childhood leukodystrophy.
  • VWM is characterized by cerebellar ataxia and neurological deterioration, triggered by stress.
  • Mutations in eukaryotic translation initiation factor 2B (eIF2B) genes cause VWM.

Purpose of the Study:

  • To elucidate the molecular mechanisms underlying VWM pathogenesis.
  • To investigate the role of astrocytes in VWM.
  • To understand the broader implications of translation defects in leukodystrophies.

Main Methods:

  • Analysis of clinical, pathological, and molecular features of VWM.
  • Investigation of eIF2B gene mutations and their functional consequences.
  • Examination of astrocyte and oligodendrocyte pathology in VWM brains.

Main Results:

  • VWM pathology involves white matter rarefaction, cystic degeneration, and immature astrocytes.
  • Astrocytes play a central role in VWM brain pathology, affecting oligodendroglia and axons.
  • Disease mechanisms include unfolded protein response activation and impaired astrocyte-microglia crosstalk.

Conclusions:

  • VWM is an astrocytopathy characterized by loss and gain of detrimental astrocytic functions.
  • Defects in mRNA translation represent a common mechanism in growing leukodystrophy group.
  • VWM serves as a model for understanding white matter biology and pathology.