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Updated: Jun 12, 2025

Fiber Connections of the Supplementary Motor Area Revisited: Methodology of Fiber Dissection, DTI, and Three Dimensional Documentation

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Vanishing white matter.

Marjo S van der Knaap¹, Marianna Bugiani², Truus E M Abbink³

¹Department of Child Neurology, Amsterdam Leukodystrophy Center, Emma Children's Hospital, Amsterdam University Medical Center, and Amsterdam Neuroscience, Cellular & Molecular Mechanisms, Vrije Universiteit, Amsterdam, The Netherlands.

Handbook of Clinical Neurology

|September 25, 2024

Summary

This summary is machine-generated.

Vanishing white matter (VWM) is a rare leukodystrophy caused by eIF2B gene variants. Targeting eIF2B pathways and the integrated stress response in astrocytes may offer new therapeutic strategies for VWM.

Keywords:

Astrocytes Eukaryotic initiation factor 2B (eIF2B)Integrated stress response (ISR)Oligodendrocytes Ovarian failure Vanishing white matter (VWM)

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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases

Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases

Published on: July 28, 2013

Area of Science:

Neuroscience
Genetics
Cell Biology

Background:

Vanishing white matter (VWM) is a severe leukodystrophy with variable onset and progression.
It results from pathogenic variants in eukaryotic initiation factor 2B (eIF2B) genes, affecting white matter and astrocytes.
The selective vulnerability of the central nervous system white matter and ovaries remains poorly understood.

Purpose of the Study:

To investigate the role of eIF2B in the integrated stress response (ISR) in VWM.
To explore the specific activation of ISR in astrocytes in VWM pathogenesis.
To identify potential therapeutic targets by modulating eIF2B activity and ISR.

Main Methods:

Analysis of VWM pathogenesis linked to eIF2B dysfunction.
Investigation of ISR activation in astrocytes in VWM models.
Assessment of therapeutic potential in VWM mouse models.

Main Results:

Genetic variants in eIF2B lead to decreased activity and constitutive ISR activation.
ISR is specifically activated in astrocytes in VWM.
Modulating eIF2B activity and ISR impacts disease severity in VWM mouse models.

Conclusions:

eIF2B is a critical regulator of the ISR, and its dysfunction drives VWM pathogenesis.
Targeting eIF2B-regulated pathways and ISR in astrocytes presents a promising therapeutic avenue for VWM.