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Lipoprotein receptor‐related protein 4 autoantibodies in myasthenia gravis: Where are we and where are we going?
Author(s) -
Nakane Shunya,
Higuchi Osamu,
Takamatsu Koutaro,
Mukaino Akihiro,
Maeda Yasuhiro,
Matsuo Hidenori,
Ando Yukio
Publication year - 2019
Publication title -
clinical and experimental neuroimmunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.297
H-Index - 15
ISSN - 1759-1961
DOI - 10.1111/cen3.12519
Subject(s) - agrin , myasthenia gravis , autoantibody , acetylcholine receptor , pathogenesis , neuromuscular junction , endocrinology , immunology , receptor , medicine , muscle weakness , antibody , biology , neuroscience
Myasthenia gravis ( MG ) is an autoimmune disorder of the neuromuscular junction that leads to fatigue of skeletal muscles and fluctuating weakness. The pathogenesis of MG is sufficiently diverse to include autoimmune mechanisms. In most patients with MG , autoantibodies against acetylcholine receptor can be detected. In a smaller proportion of patients without anti‐acetylcholine receptor antibodies, autoantibodies to muscle‐specific kinase and low‐density lipoprotein receptor‐related protein 4 ( LRP 4) are present. LRP 4 is critical for neuromuscular junction formation and maintenance. A multimolecular complex is formed among agrin, LRP 4 and muscle‐specific kinase that is critical for agrin signaling. The presence of anti‐ LRP 4 antibodies is associated with mild symptoms of MG , while thymomas are rarely observed in association with MG and LRP 4 antibodies. We review recent findings on the pathogenesis, clinical features and treatment of LRP 4 MG .