2025
Permissive central tolerance plus defective peripheral checkpoints license pathogenic memory B cells in CASPR2-antibody encephalitis
Sun B, Fernandes D, Soltys J, Kienzler A, Paneva S, Harrison R, Ramanathan S, Harrison A, Makuch M, Fichtner M, Donat R, Akdeniz D, Bayuangga H, Im M, Williams R, Vasconcelos A, Thomsen S, Fower A, Sun R, Fox H, Mgbachi V, Davies A, Tseng M, Handel A, Kelly M, Zhao M, Bancroft J, Bashford-Rogers R, Pluvinage J, Dandekar R, Alvarenga B, Dustin L, Rinaldi S, Owens R, Anthony D, Bennett D, Waters P, Davis S, Wilson M, O’Connor K, Carvalho A, Irani S. Permissive central tolerance plus defective peripheral checkpoints license pathogenic memory B cells in CASPR2-antibody encephalitis. Science Advances 2025, 11: eadr9986. PMID: 40238887, PMCID: PMC12002137, DOI: 10.1126/sciadv.adr9986.Peer-Reviewed Original ResearchConceptsMemory B cellsB cell receptorB cellsCentral toleranceDefective peripheral toleranceAutoantibody-mediated diseasesNaive B cellsGerminal center reactionImmune checkpointsPeripheral toleranceCenter reactionSomatic mutationsNeuronal culturesPathogenic effectsPatientsReceptorsEncephalitisDiseaseAutoreactivitiesAutoantibodiesAutoimmunityCaspr2ImmunobiologyAutoantigensTolerance
2013
CVID-associated TACI mutations affect autoreactive B cell selection and activation
Romberg N, Chamberlain N, Saadoun D, Gentile M, Kinnunen T, Ng YS, Virdee M, Menard L, Cantaert T, Morbach H, Rachid R, Martinez-Pomar N, Matamoros N, Geha R, Grimbacher B, Cerutti A, Cunningham-Rundles C, Meffre E. CVID-associated TACI mutations affect autoreactive B cell selection and activation. Journal Of Clinical Investigation 2013, 123: 4283-4293. PMID: 24051380, PMCID: PMC3786721, DOI: 10.1172/jci69854.Peer-Reviewed Original ResearchMeSH KeywordsAdultAntibodies, AntinuclearAutoantibodiesB-Cell Activating FactorB-LymphocytesCase-Control StudiesCell ProliferationCells, CulturedCentral ToleranceCommon Variable ImmunodeficiencyDNA-Binding ProteinsGene DosageHeterozygoteHumansLymphocyte ActivationMaleMiddle AgedMutationPeripheral ToleranceProto-Oncogene Proteins c-bcl-6T-Lymphocytes, Helper-InducerToll-Like Receptor 7Toll-Like Receptor 9Transmembrane Activator and CAML Interactor ProteinConceptsCommon variable immune deficiencyB cell activationAutoreactive B cellsTACI mutationsCell activationAntinuclear antibodiesCVID patientsB cellsFollicular helper cellsCentral B cell toleranceVariable immune deficiencyB cell responsivenessB-cell lymphoma 6B cell toleranceTumor necrosis factor receptorNecrosis factor receptorB cell selectionAutoimmune complicationsAutoimmune manifestationsPrimary diseaseAntibody deficiencyImmune toleranceHelper cellsTolerance checkpointsImmune deficiency
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