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Brief Communications
Autoantibodies to low-density lipoprotein receptor–related protein 4 in myasthenia gravis
Article first published online: 8 MAR 2011
DOI: 10.1002/ana.22312
Copyright © 2011 American Neurological Association
Additional Information
How to Cite
Higuchi, O., Hamuro, J., Motomura, M. and Yamanashi, Y. (2011), Autoantibodies to low-density lipoprotein receptor–related protein 4 in myasthenia gravis. Ann Neurol., 69: 418–422. doi: 10.1002/ana.22312
Publication History
- Issue published online: 8 MAR 2011
- Article first published online: 8 MAR 2011
- Accepted manuscript online: 28 OCT 2010 07:27AM EST
- Manuscript Accepted: 15 OCT 2010
- Manuscript Revised: 28 SEP 2010
- Manuscript Received: 4 MAY 2010
Abstract
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction, where acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein (LDL) receptor-related protein 4 (Lrp4) are essential. About 80% and 0% to 10% of patients with generalized MG have autoantibodies to AChR and MuSK, respectively, but pathogenic factors are elusive in others. Here we show that a proportion of AChR antibody-negative patients have autoantibodies to Lrp4. These antibodies inhibit binding of Lrp4 to its ligand and predominantly belong to the immunoglobulin G1 (IgG1) subclass, a complement activator. These findings together indicate the involvement of Lrp4 antibodies in the pathogenesis of AChR antibody-negative MG. Ann Neurol 2011