Premium
Characteristics of myasthenia gravis with antibodies to muscle‐specific kinase and low‐density lipoprotein‐related receptor protein 4
Author(s) -
Evoli Amelia,
Iorio Raffaele
Publication year - 2015
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/cen3.12173
Subject(s) - agrin , myasthenia gravis , acetylcholine receptor , neuromuscular junction , receptor , antibody , autoantibody , endocrinology , chemistry , biology , medicine , immunology , neuroscience
Myasthenia gravis ( MG ) is characterized by fatigable weakness of voluntary muscles caused by immunoglobulin G autoantibodies binding to post‐synaptic proteins at the neuromuscular junction. Antibodies specific for the acetylcholine receptor are detected in the great majority (85%) of MG patients. Serum immunoglobulin G binding to the muscle‐specific kinase (Mu SK ) are found in approximately 40% of anti‐acetylcholine receptor‐negative patients. Recently, the low‐density lipoprotein receptor‐related protein 4 (Lrp4), which is the Mu SK co‐receptor for agrin, has been recognized as a new antigen in MG . Specific antibodies bind to the extracellular domains of Mu SK and Lrp4 and, as experimental models have shown, interfere with the protein function. The present review describes the Mu SK ‐Lrp4 complex, and summarizes the effects of Mu SK and Lrp4 antibodies at the neuromuscular junction. Furthermore, the epidemiological and clinical characteristics, the response to therapy and the controversies in management of the specific diseases are discussed.