1. Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome.
- Author
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Huijbers MG, Lipka AF, Potman M, Hensbergen PJ, Titulaer MJ, Niks EH, van der Maarel SM, Klooster R, and Verschuuren JJ
- Subjects
- Adult, Autoantibodies blood, Autoantigens isolation & purification, Calcium Channels immunology, Cell Line, Humans, Lambert-Eaton Myasthenic Syndrome complications, Lung Neoplasms complications, Male, Small Cell Lung Carcinoma complications, Adaptor Proteins, Signal Transducing immunology, Autoantigens immunology, Lambert-Eaton Myasthenic Syndrome immunology, Lung Neoplasms immunology, Nerve Tissue Proteins immunology, Small Cell Lung Carcinoma immunology
- Abstract
Lambert-Eaton myasthenic syndrome (LEMS) is characterized by fluctuating muscle weakness and autonomic dysfunction. In 90% of the LEMS patients the disease is associated with auto-antibodies against the voltage-gated calcium channels (VGCC). Several auto-immune responses against other antigenic targets have been described to (co)-occur in LEMS patients. To identify new LEMS associated small cell lung cancer (SCLC) markers immunoprecipitation with a SCLC cell line was performed. We discovered strong immunoreactivity against the 120 kDa large ERC1 protein in one tumor-negative VGCC-positive LEMS patient. A recombinant ELISA assay and a cellular assay expressing GFP-tagged full length ERC1 were used to confirm the presence of auto-antibodies against ERC1 in this patient. Additional testing of 58 LEMS patients including 9 VGCC auto-antibody negative LEMS patients, 48 myasthenia gravis patients, 84 control patients with other diseases and 12 healthy controls revealed no other cases. ERC1 is therefore a new, but rare, antigen in LEMS., (Copyright © 2013 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)
- Published
- 2013
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