Web of Science: 2 citations, Scopus: 2 citations, Google Scholar: citations,
Characterization of an anti-fetal AChR monoclonal antibody isolated from a myasthenia gravis patient
Saxena, Abhishek (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Stevens, Jo (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Cetin,Hakan (Nuffield Department of Clinical Neurosciences, John Radcliffe University Hospital, Oxford)
Koneczny, Inga (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Webster, Richard (Nuffield Department of Clinical Neurosciences, John Radcliffe University Hospital, Oxford)
Lazaridis, Konstantinos (Hellenic Pasteur Institute)
Tzartos, Socrates (Hellenic Pasteur Institute)
Vrolix, Kathleen (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Nogales-Gadea, Gisela (Institut Germans Trias i Pujol. Recerca Neuromuscular i Neuropediàtrica)
Machiels, Barbie (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Molenaar, Peter C. (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Damoiseaux, Jan (Central Diagnostic Laboratory, Maastricht University Medical Center)
De Baets, Marc H. (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Simon-Keller, Katja (Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg)
Marx, Alexander (Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg)
Vincent, Angela (Nuffield Department of Clinical Neurosciences, John Radcliffe University Hospital, Oxford)
Mario Losen (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)
Pilar Martinez-Martinez (Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University)

Date: 2017
Abstract: We report here the sequence and functional characterization of a recombinantly expressed.
Abstract: autoantibody (mAb 131) previously isolated from a myasthenia gravis patient by immortalization of thymic B cells using Epstein-Barr virus and TLR9 activation. The antibody is characterized by a high degree of somatic mutations as well as a 6 amino acid insertion within the VHCDR2. The recombinant mAb 131 is specific for the γ-subunit of the fetal AChR to which it bound with sub-nanomolar apparent affinity, and detected the presence of fetal AChR on a number of rhabdomyosarcoma cell lines. Mab 131 blocked one of the two α-bungarotoxin binding sites on the fetal AChR, and partially blocked the binding of an antibody (mAb 637) to the α-subunit of the AChR, suggesting that both antibodies bind at or near one ACh binding site at the α/γ subunit interface. However, mAb 131 did not reduce fetal AChR ion channel currents in electrophysiological experiments. These results indicate that mAb 131, although generated from an MG patient, is unlikely to be pathogenic and may make it a potentially useful reagent for studies of myasthenia gravis, rhabdomyosarcoma and arthrogryposis multiplex congenita which can be caused by fetal-specific AChR-blocking autoantibodies.
Note: Número d'acord de subvenció ISCIII/CP14/00032
Note: Número d'acord de subvenció FIS/PI15/01756
Rights: Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. Creative Commons
Language: Anglès.
Document: article ; recerca ; publishedVersion
Published in: Sientific reports, Vol. 7 (october 2017) , p. 1-12

DOI: 10.1038/s41598-017-14350-8
PMID: 29089519


12 p, 5.0 MB

The record appears in these collections:
Research literature > UAB research groups literature > Research Centres and Groups (scientific output) > Health sciences and biosciences > Institut d'Investigació en Ciencies de la Salut Germans Trias i Pujol (IGTP)
Articles > Research articles
Articles > Published articles

 Record created 2018-10-26, last modified 2019-03-01



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