Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome

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

doi:10.1016/j.humimm.2013.03.004

Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome

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

^*Corresponding author for this work

Leiden University Medical Centre

Research output: Contribution to journal › Article › Academic › peer-review

8 Citations (Scopus)

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.

Original language	English
Pages (from-to)	849-851
Number of pages	3
Journal	Human Immunology
Volume	74
Issue number	7
DOIs	https://doi.org/10.1016/j.humimm.2013.03.004
Publication status	Published - Jul 2013
Externally published	Yes

Bibliographical note

Funding Information:
We kindly thank Ingrid Hegeman and Irina Dragan for their technical support. Part of this research was supported by the Prinses Beatrix Spierfonds, Koningin Wilhelmina Foundation, and Association Française contre les Myopathies .

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SDG 3 Good Health and Well-being

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@article{4b4350dde8ae400eb00f092c2cd60036,

title = "Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome",

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.",

author = "Huijbers, \{Maartje G.\} and Lipka, \{Alexander F.\} and Marko Potman and Hensbergen, \{Paul J.\} and Titulaer, \{Maarten J.\} and Niks, \{Erik H.\} and \{van der Maarel\}, \{Silv{\`e}re M.\} and Rinse Klooster and Verschuuren, \{Jan J.\}",

note = "Funding Information: We kindly thank Ingrid Hegeman and Irina Dragan for their technical support. Part of this research was supported by the Prinses Beatrix Spierfonds, Koningin Wilhelmina Foundation, and Association Fran{\c c}aise contre les Myopathies .",

year = "2013",

month = jul,

doi = "10.1016/j.humimm.2013.03.004",

language = "English",

volume = "74",

pages = "849--851",

journal = "Human Immunology",

issn = "0198-8859",

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T1 - Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome

AU - Huijbers, Maartje G.

AU - Lipka, Alexander F.

AU - Potman, Marko

AU - Hensbergen, Paul J.

AU - Titulaer, Maarten J.

AU - Niks, Erik H.

AU - van der Maarel, Silvère M.

AU - Klooster, Rinse

AU - Verschuuren, Jan J.

N1 - Funding Information: We kindly thank Ingrid Hegeman and Irina Dragan for their technical support. Part of this research was supported by the Prinses Beatrix Spierfonds, Koningin Wilhelmina Foundation, and Association Française contre les Myopathies .

PY - 2013/7

Y1 - 2013/7

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/84878573294

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DO - 10.1016/j.humimm.2013.03.004

M3 - Article

C2 - 23583364

AN - SCOPUS:84878573294

SN - 0198-8859

VL - 74

SP - 849

EP - 851

JO - Human Immunology

JF - Human Immunology

IS - 7

ER -

Antibodies to active zone protein ERC1 in Lambert-Eaton myasthenic syndrome

Abstract

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