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.
|
Authors | Maartje G Huijbers, Alexander F Lipka, Marko Potman, Paul J Hensbergen, Maarten J Titulaer, Erik H Niks, Silvère M van der Maarel, Rinse Klooster, Jan J Verschuuren |
Journal | Human immunology
(Hum Immunol)
Vol. 74
Issue 7
Pg. 849-51
(Jul 2013)
ISSN: 1879-1166 [Electronic] United States |
PMID | 23583364
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Copyright | Copyright © 2013 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved. |
Chemical References |
- Adaptor Proteins, Signal Transducing
- Autoantibodies
- Autoantigens
- Calcium Channels
- ERC1 protein, human
- Nerve Tissue Proteins
|
Topics |
- Adaptor Proteins, Signal Transducing
(immunology)
- Adult
- Autoantibodies
(blood)
- Autoantigens
(immunology, isolation & purification)
- Calcium Channels
(immunology)
- Cell Line
- Humans
- Lambert-Eaton Myasthenic Syndrome
(complications, immunology)
- Lung Neoplasms
(complications, immunology)
- Male
- Nerve Tissue Proteins
(immunology)
- Small Cell Lung Carcinoma
(complications, immunology)
|