title: Antibodies against Wnt receptor of muscle-specific tyrosine kinase in myasthenia gravis.
abstract: Muscle-specific tyrosine kinase (MuSK) antibodies are detected in a proportion of myasthenia gravis (MG) patients who are negative for acetylcholine receptor (AChR) antibodies and have prominent bulbar weakness and crises. In the MuSK ectodomains, the immunoglobulin-like 1 and 2 domains (Ig1/2) mediate the agrin-Lrp4-MuSK signaling and the cysteine-rich domain (CRD) mediates the Wnt-MuSK-Dishevelled signaling; both contribute to AChR clustering. Immunoblotting against recombinant proteins showed MuSK Ig1/2 antibodies in 33 anti-AChR-negative MG patients; 10 patients of them (30%) were additionally positive for MuSK CRD antibodies. The result suggests that MuSK antibodies have heterogeneity in their binding to functional domains of MuSK.
authors.affiliation: Neurological Center, Kanazawa-Nishi Hospital, 6-15-41, Ekinishi-Honmachi, Kanazawa, Ishikawa-ken 920-0025, Japan. m-takamori@vanilla.ocn.ne.jp
authors.forename: Masaharu
authors.initials: M
authors.lastname: Takamori
authors.forename: Tatsufumi
authors.initials: T
authors.lastname: Nakamura
authors.forename: Masakatsu
authors.initials: M
authors.lastname: Motomura
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000378:metabolism; D002648:Child; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000378:metabolism* / Q000503:physiopathology; D020794:Receptor Protein-Tyrosine Kinases / Q000378:metabolism*; D011950:Receptors, Cholinergic / Q000378:metabolism*; D060490:Receptors, Wnt / Q000276:immunology*; D055815:Young Adult
chemical_list: D000906:Antibodies; D011950:Receptors, Cholinergic; D060490:Receptors, Wnt; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases