Impaired B Cell Expression of the Inhibitory Fcγ Receptor IIB in Myasthenia Gravis

Language
en
Document Type
Article
Issue Date
2022-11-23
First published
2022-11-16
Issue Year
2022
Authors
Keller, Christian W.
Chuquisana, Omar
Derdelinckx, Judith
Gross, Catharina C.
Berger, Klaus
Robinson, James
Nimmerjahn, Falk
Wiendl, Heinz
Willcox, Nick
Lünemann, Jan D.
Editor
Publisher
John Wiley & Sons, Inc.
Abstract

Myasthenia gravis (MG) is an autoimmune disease in which pathogenic immunoglobulin G antibodies bind to acetylcholine receptors (or to functionally related molecules at the neuromuscular junction). B cell expression of the inhibitory immunoglobulin G receptor, Fc‐gamma receptor (FcγR) IIB, maintains peripheral immune tolerance, and its absence renders B cells hyperresponsive to autoantigen. Here, we report that FcγRIIB expression levels are substantially reduced in B lineage cells derived from immunotherapy‐naïve patients with acetylcholine receptor antibody‐positive early‐onset MG. In contrast, genetic variants associated with impaired FcγRIIB expression are not enriched in MG, indicating post‐transcriptional dysregulation. FcγR‐targeted therapies could have therapeutic benefits in MG. ANN NEUROL 2022;92:1046–1051

Journal Title
Annals of Neurology
Volume
92
Issue
6
Citation

Annals of Neurology 92.6 (2022): 1046-1051. https://onlinelibrary.wiley.com/doi/full/10.1002/ana.26507

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