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Updated: Sep 6, 2025

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
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Published on: October 21, 2014

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Myelin oligodendrocyte glycoprotein antibodies in genetic leukodystrophies

Nesibe Gevher Eroglu-Ertugrul1, Mohammadreza Yousefi2, Faruk Pekgül3

  • 1Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Journal of Neuroimmunology
|June 25, 2022
PubMed

Insights

Genetic leukodystrophies may involve autoimmune processes. Researchers detected anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in some patients with metachromatic leukodystrophy (MLD) and vanishing white matter (VWM) disease.

Area of Science:

  • Neuroimmunology
  • Genetics
  • Metabolic Disorders

Background:

  • Genetic leukodystrophies are characterized by demyelination and inflammation.
  • Accumulation of intermediate metabolites contributes to disease pathogenesis.

Purpose of the Study:

  • To investigate the presence of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with genetic leukodystrophies.
  • To explore a potential autoimmune component in these inherited neurological disorders.

Main Methods:

  • Sera from 30 patients with genetic leukodystrophy and 48 healthy controls were analyzed.
  • Anti-MOG antibodies were detected using fixed and/or live cell-based assays.

Main Results:

  • Anti-MOG IgG antibodies were detected in two patients with late infantile metachromatic leukodystrophy (MLD).
  • One MLD patient showed IgG1 and IgG3 subclasses, while the other showed dominant IgG3.
  • Borderline positive anti-MOG IgG was found in one patient with vanishing white matter (VWM) disease.

Conclusions:

  • Inherited metabolic or degenerative processes in leukodystrophies may be associated with an autoimmune component.
  • The presence of anti-MOG antibodies might represent an epiphenomenon in these conditions.
Keywords:
DemyelinationFixed cell-based assayLeukodystrophyLive cell-based assayMOG antibody disease (MOGAD)Myelin oligodendrocyte glycoprotein

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