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Autoantibodies to low‐density lipoprotein receptor‐related protein 4 that is essential for the neuromuscular junction formation
Author(s) -
Higuchi Osamu
Publication year - 2013
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/cen3.12020
Subject(s) - autoantibody , myasthenia gravis , neuromuscular junction , acetylcholine receptor , neuromuscular transmission , postsynaptic potential , antibody , pathogenesis , agrin , muscle weakness , receptor , endocrinology , medicine , immunology , chemistry , biology , neuroscience
Abstract Myasthenia gravis ( MG ) is an autoimmune disorder characterized by skeletal muscle fatigability and muscle weakness resulting from inhibition of neuromuscular transmission by autoantibodies against synaptic apparatus in the neuromuscular junction ( NMJ ). MG is probably classified into three groups: anti‐acetylcholine receptor ( AC hR) antibody‐positive MG ; antimuscle‐specific kinase (Mu SK ) antibody‐positive MG ; and seronegative MG , which is negative for both antibodies. In patients with seronegative MG , pathogenic factors remain elusive. Recently, a new autoantibody against low‐density lipoprotein receptor‐related protein 4 (Lrp4), which is one of the postsynaptic apparatus and indispensable for the formation of the NMJ , has been identified and has attracted attention as a third MG ‐related autoantibody. In the present review, we discuss the role of Lrp4 in the formation of NMJ , and the molecular mechanism(s) of pathogenesis for anti‐Lrp4 antibody‐positive MG .