PMID- 21387385 OWN - NLM STAT- MEDLINE DCOM- 20110511 LR - 20220408 IS - 1531-8249 (Electronic) IS - 0364-5134 (Linking) VI - 69 IP - 2 DP - 2011 Feb TI - Autoantibodies to low-density lipoprotein receptor-related protein 4 in myasthenia gravis. PG - 418-22 LID - 10.1002/ana.22312 [doi] AB - 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. CI - Copyright (c) 2011 American Neurological Association. FAU - Higuchi, Osamu AU - Higuchi O AD - Division of Genetics, Department of Cancer Biology, the Institute of Medical Science, the University of Tokyo, Japan. FAU - Hamuro, Johko AU - Hamuro J FAU - Motomura, Masakatsu AU - Motomura M FAU - Yamanashi, Yuji AU - Yamanashi Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Neurol JT - Annals of neurology JID - 7707449 RN - 0 (Autoantibodies) RN - 0 (Immunoglobulin G) RN - 0 (LDL-Receptor Related Proteins) RN - 0 (Receptors, Cholinergic) SB - IM MH - Autoantibodies/*immunology MH - Humans MH - Immunoglobulin G/immunology MH - LDL-Receptor Related Proteins/*immunology MH - Myasthenia Gravis/*immunology MH - Radioimmunoprecipitation Assay MH - Receptors, Cholinergic/immunology EDAT- 2011/03/10 06:00 MHDA- 2011/05/12 06:00 CRDT- 2011/03/10 06:00 PHST- 2011/03/10 06:00 [entrez] PHST- 2011/03/10 06:00 [pubmed] PHST- 2011/05/12 06:00 [medline] AID - 10.1002/ana.22312 [doi] PST - ppublish SO - Ann Neurol. 2011 Feb;69(2):418-22. doi: 10.1002/ana.22312.