Lambert-Eaton myasthenic syndrome as an autoimmune calcium channelopathy.
Lambert-Eaton myasthenic syndrome, often associated with small-cell lung carcinoma, is a disease of neuromuscular transmission in which antibodies directed against voltage-gated calcium channel (VGCC)(P/Q-type) in the motor nerve terminal play a crucial role in causing a deficient quantal release of acetylcholine. The motor nerve terminal and carcinoma cell may share a common antigen. The study using synthetic peptides and recombinant protein specified the extracellular S5-S6 linker regions in 3 of 4 domains as immunodominant sites in the molecular structure of P/Q-type VGCC alpha1 subunit. Also, the study by use of peptides and recombinant protein corresponding to synaptotagmin I suggested that in this functionally VGCC-associated presynaptic protein, the segment which exposes extracellularly during exocytosis can be immunogenic for the syndrome.
Takamori
Masaharu
M
Neurological Center, Kanazawa-Nishi Hospital and Kanazawa University, Kanazawa, Japan. t-kiyomi@guitar.ocn.ne.jp
D000818:Animals; D015220:Calcium Channels / Q000276:immunology; D020865:Calcium Channels, P-Type / Q000737:chemistry / Q000276:immunology*; D020907:Calcium Channels, Q-Type / Q000737:chemistry / Q000276:immunology*; D002135:Calcium-Binding Proteins / Q000276:immunology; D004195:Disease Models, Animal; D018604:Epitope Mapping; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000276:immunology*; D008562:Membrane Glycoproteins / Q000276:immunology; D051379:Mice; D009419:Nerve Tissue Proteins / Q000276:immunology; D051381:Rats; D050863:Synaptotagmin I; D050857:Synaptotagmins
D015220:Calcium Channels; D020865:Calcium Channels, P-Type; D020907:Calcium Channels, Q-Type; D002135:Calcium-Binding Proteins; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; C494310:SYT1 protein, human; D050863:Synaptotagmin I; C494311:Syt1 protein, mouse; C494312:Syt1 protein, rat; D050857:Synaptotagmins