Antibodies to calcium channel and synaptotagmin in Lambert-Eaton myasthenic syndrome.
In the Lambert-Eaton myasthenic syndrome (LEMS), an autoimmune disease that is often associated with lung cancer and characterized by reduced quantal release of acetylcholine from the motor nerve terminal, our studies to search for the target of LEMS antibodies have brought the voltage-gated calcium channel (VGCC) into relief. Among multiple types of VGCCs, the P/Q-type was highly recognized by LEMS antibodies. Using synthetic peptides or recombinant proteins as antigens, the study specified the S5-S6 linker regions in 3 of 4 domains as immunodominant sites in the molecular structure of P/Q-type VGCC alpha1 subunit. Synaptotagmin, one of the functionally VGCC-associated synaptic proteins, was also found to be an immunogen in the pathogenesis of LEMS.
Takamori
M
M
Neurological Center, Kanazawa-Nishi Hospital, Kanazawa, Japan.
Komai
K
K
Iwasa
K
K
D001323:Autoantibodies / Q000378:metabolism*; D015220:Calcium Channels / Q000276:immunology*; D002135:Calcium-Binding Proteins / Q000276:immunology*; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000276:immunology* / Q000378:metabolism; D008562:Membrane Glycoproteins / Q000276:immunology*; D009419:Nerve Tissue Proteins / Q000276:immunology*; D009469:Neuromuscular Junction / Q000276:immunology*; D020361:Paraneoplastic Syndromes, Nervous System / Q000276:immunology; D011956:Receptors, Cell Surface / Q000276:immunology*; D050857:Synaptotagmins
D001323:Autoantibodies; D015220:Calcium Channels; D002135:Calcium-Binding Proteins; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; D011956:Receptors, Cell Surface; D050857:Synaptotagmins