Autoantibodies to low‐density lipoprotein receptor–related protein 4 in myasthenia gravis
O Higuchi, J Hamuro, M Motomura… - Annals of …, 2011 - Wiley Online Library
O Higuchi, J Hamuro, M Motomura, Y Yamanashi
Annals of neurology, 2011•Wiley Online LibraryMyasthenia 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 …
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 …
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
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