Autoantibodies to low-density lipoprotein receptor-related protein 4 that is essential for the neuromuscular junction formation

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: an...

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Bibliographic Details
Published in:Clinical & experimental neuroimmunology 2013-08, Vol.4 (2), p.216-219
Main Author: Higuchi, Osamu
Format: Article
Language:English
Subjects:
autoantibody
Autoimmune diseases
Cholesterol
Muscular system
myasthenia gravis
neuromuscular junction
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Summary: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 (AChR) antibody‐positive MG; antimuscle‐specific kinase (MuSK) 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.
ISSN:1759-1961
1759-1961
DOI:10.1111/cen3.12020