title: Immunoproteomic studies on paediatric opsoclonus-myoclonus associated with neuroblastoma.
abstract: We aimed to identify new cell-membrane antigens implicated in opsoclonus-myoclonus with neuroblastoma. The sera of 3 out of 14 patients showed IgG electron-microscopy immunogold reactivity on SH-SY5Y neuroblastoma cells. Immunoprecipitation experiments using rat brain synaptosomes and SH-SY5Y cells led to the identification of: (1) thirty-one nuclear/cytoplasmic proteins (including antigens HuB, HuC); (2) seven neuronal membrane proteins, including the Shaw-potassium channel Kv3.3 (KCNC3), whose genetic disruption in mice causes ataxia and generalized muscle twitching. Although cell-based assays did not demonstrate direct antigenicity, our findings point to Shaw-related subfamily of the potassium voltage-gated channels complexed proteins as hypothetical antigenic targets.
authors.affiliation: Laboratorio de Neurología, Instituto de Investigación Sanitaria La Fe, Hospital Universitario y Politécnico La Fe, CIBERER, Valencia, Spain.
authors.forename: Estefanía
authors.initials: E
authors.lastname: Torres-Vega
authors.affiliation: Laboratorio de Neurobiología Comparada, Instituto Cavanilles, Universidad de Valencia, CIBERNED, Valencia, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Durán-Moreno
authors.affiliation: Departamento de Bioquímica y Biología Molecular, Universidad de Valencia, Valencia, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Sánchez Del Pino
authors.affiliation: Unidad de Oncología Pediátrica, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors.forename: Yania
authors.initials: Y
authors.lastname: Yáñez
authors.affiliation: Unidad de Oncología Pediátrica, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors.forename: Adela
authors.initials: A
authors.lastname: Cañete
authors.affiliation: Unidad de Oncología Pediátrica, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors.forename: Victoria
authors.initials: V
authors.lastname: Castel
authors.affiliation: Servicio de Neurología, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors.forename: Rogelio
authors.initials: R
authors.lastname: López-Cuevas
authors.affiliation: Laboratorio de Neurología, Instituto de Investigación Sanitaria La Fe, Hospital Universitario y Politécnico La Fe, CIBERER, Valencia, Spain; Servicio de Neurología, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors.forename: Juan Jesús
authors.initials: JJ
authors.lastname: Vílchez
authors.affiliation: Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA, and Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain; Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain; Laboratori de Neurologia, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), and Servei de Neurologia, Hospital Clínic, Barcelona, Spain.
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
authors.affiliation: Laboratori de Neurologia, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), and Servei de Neurologia, Hospital Clínic, Barcelona, Spain.
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.affiliation: Laboratorio de Neurobiología Comparada, Instituto Cavanilles, Universidad de Valencia, CIBERNED, Valencia, Spain.
authors.forename: José Manuel
authors.initials: JM
authors.lastname: García Verdugo
authors.affiliation: Laboratorio de Neurología, Instituto de Investigación Sanitaria La Fe, Hospital Universitario y Politécnico La Fe, CIBERER, Valencia, Spain; Servicio de Neurología, Hospital Universitario y Politécnico La Fe, Valencia, Spain. Electronic address: bataller_lui@gva.es.
authors.forename: Luis
authors.initials: L
authors.lastname: Bataller
mesh_terms: D000818:Animals; D001932:Brain Neoplasms / Q000150:complications / Q000276:immunology / Q000473:pathology; D015816:Cell Adhesion Molecules, Neuronal / Q000378:metabolism; D045744:Cell Line, Tumor; D002490:Central Nervous System / Q000378:metabolism* / Q000648:ultrastructure; D002648:Child; D016208:Databases, Factual / Q000706:statistics & numerical data; D004660:Encephalitis / Q000150:complications / Q000276:immunology; D005260:Female; D057809:HEK293 Cells; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000378:metabolism / Q000648:ultrastructure; D009447:Neuroblastoma / Q000150:complications / Q000276:immunology / Q000473:pathology; D009687:Nuclear Proteins / Q000378:metabolism / Q000648:ultrastructure; D053578:Opsoclonus-Myoclonus Syndrome / Q000150:complications / Q000276:immunology / Q000473:pathology; D051381:Rats; D017208:Rats, Wistar; D051670:Shaw Potassium Channels / Q000276:immunology / Q000378:metabolism / Q000648:ultrastructure; D013574:Synaptosomes / Q000378:metabolism / Q000648:ultrastructure; D013945:Thymoma / Q000150:complications
chemical_list: D015816:Cell Adhesion Molecules, Neuronal; C000591569:IgLON5 protein, human; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D051670:Shaw Potassium Channels
keywords: Immunoproteomics; Opsoclonus-myoclonus; Potassium channels; Synaptosomes