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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. Copyright © 2022. Published by Elsevier B.V.

Citation

Nesibe Gevher Eroglu-Ertugrul, Mohammadreza Yousefi, Faruk Pekgül, Tansu Doran, Ceren Günbey, Meral Topcu, Kader K Oguz, Hatice Asuman Ozkara, Atay Vural, Banu Anlar. Myelin oligodendrocyte glycoprotein antibodies in genetic leukodystrophies. Journal of neuroimmunology. 2022 Aug 15;369:577916

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PMID: 35752102

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