Published January 1, 2022 | Version v1
Journal article Open

Myelin oligodendrocyte glycoprotein antibodies in genetic leukodystrophies

  • 1. Hacettepe Univ, Fac Med, Dept Pediat Neurol, Ankara, Turkey
  • 2. Koc Univ, Res Ctr Translat Med, Sch Med, Istanbul, Turkey
  • 3. Hacettepe Univ, Fac Med, Dept Med Biochem, Ankara, Turkey
  • 4. Hacettepe Univ, Fac Med, Dept Radiol, Ankara, Turkey

Description

Accumulation of intermediate metabolites due to enzyme deficiencies and demyelination can provoke inflammation in genetic leukodystrophies. Thirty patients with genetic leukodystrophy and 48 healthy control sera were tested for anti-myelin oligodendrocyte glycoprotein (MOG) antibodies by fixed and/or live cell-based assays. MOG-IgG was detected in two late infantile metachromatic leukodystrophy (MLD) cases, both of which were also weakly positive for IgG1, and one with IgG3 as the dominant anti-MOG IgG subclass. MOG-IgG was borderline positive in a vanishing white matter (VWM) disease patient. These results suggest that inherited metabolic or degenerative processes can have an autoimmune component, possibly as an epiphenomenon.

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