title: Contactin-1 and Neurofascin-155/-186 Are Not Targets of Auto-Antibodies in Multifocal Motor Neuropathy.
abstract: Multifocal motor neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyneuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal motor neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal motor neuropathy patients' sera. We analyzed sera of 33 patients with well-characterized multifocal motor neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal motor neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal motor neuropathy.
authors.affiliation: Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Kathrin
authors.initials: K
authors.lastname: Doppler
authors.affiliation: Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Luise
authors.initials: L
authors.lastname: Appeltshauser
authors.affiliation: Department of Neurology, University Hospital Gießen, Gießen, Germany.
authors.forename: Heidrun H
authors.initials: HH
authors.lastname: Krämer
authors.affiliation: Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Judy King Man
authors.initials: JK
authors.lastname: Ng
authors.affiliation: Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Edgar
authors.initials: E
authors.lastname: Meinl
authors.affiliation: Institute for Clinical Neurobiology, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Carmen
authors.initials: C
authors.lastname: Villmann
authors.affiliation: Centre for Neuroregeneration, University of Edinburgh, Edinburgh, United Kingdom.
authors.forename: Peter
authors.initials: P
authors.lastname: Brophy
authors.affiliation: Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, 06510, United States of America; Center of Neuroscience and Regeneration Research, Veterans Affairs Medical Center, West Haven, Connecticut, 06516, United States of America.
authors.forename: Sulayman D
authors.initials: SD
authors.lastname: Dib-Hajj
authors.affiliation: Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, 06510, United States of America; Center of Neuroscience and Regeneration Research, Veterans Affairs Medical Center, West Haven, Connecticut, 06516, United States of America.
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Waxman
authors.affiliation: Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Weishaupt
authors.affiliation: Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Claudia
authors.initials: C
authors.lastname: Sommer
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D016022:Case-Control Studies; D015815:Cell Adhesion Molecules / Q000276:immunology*; D058967:Contactin 1 / Q000276:immunology*; D005260:Female; D057809:HEK293 Cells; D006801:Humans; D008297:Male; D008810:Mice, Inbred C57BL; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000378:metabolism; D009414:Nerve Growth Factors / Q000276:immunology*; D011115:Polyneuropathies / Q000097:blood* / Q000276:immunology / Q000473:pathology; D011446:Prospective Studies; D020033:Protein Isoforms
chemical_list: D001323:Autoantibodies; C548669:CNTN1 protein, human; D015815:Cell Adhesion Molecules; D058967:Contactin 1; C054305:NFASC protein, human; D009414:Nerve Growth Factors; D020033:Protein Isoforms