title: Pathophysiology of myasthenia gravis with antibodies to the acetylcholine receptor, muscle-specific kinase and low-density lipoprotein receptor-related protein 4.
abstract: Myasthenia gravis is caused by antibodies to the acetylcholine receptor, muscle-specific kinase, low-density lipoprotein receptor-related protein 4, or possibly yet unidentified antibodies. The mechanisms by which these antibodies interfere with the function of postsynaptic proteins include complement activation, antigenic modulation by crosslinking of the target proteins, competition with ligand binding sites, or steric hindrance which inhibits conformational changes or binding to associated proteins. Screening for auto-antibodies to different postsynaptic targets, and also for low-affinity antibodies, is contributing to a more accurate diagnosis of MG patients. Further studies into the specific pathophysiological pathways of the several MG subforms might help to develop new, more antigen specific, therapies.
authors.affiliation: Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. J.J.G.M.Verschuuren@lumc.nl
authors.forename: Jan J G M
authors.initials: JJ
authors.lastname: Verschuuren
authors.forename: Maartje G
authors.initials: MG
authors.lastname: Huijbers
authors.forename: Jaap J
authors.initials: JJ
authors.lastname: Plomp
authors.forename: Erik H
authors.initials: EH
authors.lastname: Niks
authors.forename: Peter C
authors.initials: PC
authors.lastname: Molenaar
authors.forename: Pilar
authors.initials: P
authors.lastname: Martinez-Martinez
authors.forename: Alejandro M
authors.initials: AM
authors.lastname: Gomez
authors.forename: Marc H
authors.initials: MH
authors.lastname: De Baets
authors.forename: Mario
authors.initials: M
authors.lastname: Losen
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D003167:Complement Activation; D006801:Humans; D026502:LDL-Receptor Related Proteins / Q000276:immunology; D009157:Myasthenia Gravis / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D001323:Autoantibodies; D026502:LDL-Receptor Related Proteins; C551781:LRP4 protein, human; D011950:Receptors, Cholinergic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases