6533b827fe1ef96bd1286f70

RESEARCH PRODUCT

Immunoproteomic studies on paediatric opsoclonus-myoclonus associated with neuroblastoma

Francesc GrausRogelio López-cuevasJuan J. VílchezVictoria CastelManuel M. Sánchez Del PinoEstefanía Torres-vegaMaria Duran-morenoJosep DalmauAdela CañeteLuis BatallerYania YáñezJosé Manuel García-verdugo

subject

Central Nervous SystemMale0301 basic medicineAntigenicityDatabases FactualThymomaImmunoprecipitationKCTD7Cell Adhesion Molecules NeuronalImmunologyNerve Tissue ProteinsBiologyNeuroblastoma03 medical and health sciences0302 clinical medicineAntigenCell Line TumorNeuroblastomaOpsoclonus myoclonus syndromemedicineAnimalsHumansImmunology and AllergyRats WistarChildOpsoclonus-Myoclonus SyndromeBrain NeoplasmsMembrane ProteinsNuclear ProteinsImmunogold labellingmedicine.diseaseMolecular biologyRatsHEK293 Cells030104 developmental biologyShaw Potassium ChannelsNeurologyMembrane proteinEncephalitisFemaleNeurology (clinical)030217 neurology & neurosurgerySynaptosomes

description

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.

https://doi.org/10.1016/j.jneuroim.2016.05.015