Myelin oligodendrocyte glycoprotein antibodies in genetic leukodystrophies

EROĞLU ERTUĞRUL N. G., Yousefi M., Pekguel F., Doran T., Guenbey C., TOPÇU M., ...More

JOURNAL OF NEUROIMMUNOLOGY, vol.369, 2022 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 369
  • Publication Date: 2022
  • Doi Number: 10.1016/j.jneuroim.2022.577916
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, Chemical Abstracts Core, EMBASE, MEDLINE, Psycinfo, Veterinary Science Database
  • Keywords: Leukodystrophy, Myelin oligodendrocyte glycoprotein, Fixed cell-based assay, Live cell-based assay, MOG antibody disease (MOGAD), Demyelination, METACHROMATIC LEUKODYSTROPHY, NEUROINFLAMMATION, NEUROPATHY, CHILDREN, DISEASE, CELLS
  • Hacettepe University Affiliated: Yes


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.