B-Cell Maturation and Antibody Responses in Individuals Carrying a Mutated CD19 Allele

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dc.contributor.authorArtaç, H.
dc.contributor.authorReisli, İ.
dc.contributor.authorKara, R.
dc.contributor.authorPico-Knijnenburg, I.
dc.contributor.authorAdın-Çınar, S.
dc.contributor.authorPekcan, S.
dc.contributor.authorJol-Van der Zijde, C. M.
dc.contributor.authorVan Tol, M. J. D.
dc.contributor.authorBakker-Jonges, L. E.
dc.contributor.authorVan Dongen, J. J. M.
dc.contributor.authorVan der Burg, M.
dc.contributor.authorVan Zelm, M. C.
dc.date.accessioned2020-03-26T17:47:14Z
dc.date.available2020-03-26T17:47:14Z
dc.date.issued2010
dc.departmentSelçuk Üniversitesien_US
dc.description.abstractHomozygous CD19 mutations lead to an antibody deficiency due to disruption of the CD19 complex and consequent impaired signaling by the B-cell antigen receptor. We studied the effects of heterozygous CD19 mutations on peripheral B-cell development and antibody responses in a large family with multiple consanguineous marriages. Sequence analysis of 96 family members revealed 30 carriers of the CD19 mutation. Lymphocyte subset counts were not significantly different between carriers and noncarriers in three different age groups (0-10 years; 11-18 years; adults). B cells of carriers had reduced CD19 and CD21 median expression levels, and had reduced proportions of transitional (0-10 years) and CD5(+) B cells (adults). CD19 carriers did not show clinical signs of immunodeficiency; they were well capable to produce normal serum Ig levels and had normal responses to primary and booster vaccinations. The frequency of mutated V(kappa) alleles was not affected. Heterozygous loss of CD19 causes some changes in the naive B-cell compartment, but overall in vivo B-cell maturation or humoral immunity is not affected. Many antibody deficiencies are not monogenetic, but likely caused by a combination of multiple genetic variations. Therefore, functional analyses of immune cell function should be carried out to show whether heterozygous mutations contribute to disease.en_US
dc.description.sponsorshipScientific and Technological Research Council of Turkey (TUBI-TAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK); Dutch Organization for Scientific Research (NWO/ZonMW)Netherlands Organization for Scientific Research (NWO)Netherlands Organization for Health Research and Development [916.56.107]; Erasmus University Rotterdam (EUR)en_US
dc.description.sponsorshipThe authors are indebted to Mrs S Posthumus for technical support with the Ig kappa REHMA assay and Professor H Hooijkaas and his colleagues from the immunodiagnostic laboratory at the Erasmus MC for technical support with anti-nuclear antibody testing. This work was supported by a grant from the Scientific and Technological Research Council of Turkey (TUBI-TAK) to H Artac, a VENI grant (916.56.107) from the Dutch Organization for Scientific Research (NWO/ZonMW) to M van der Burg, and a grant from the Erasmus University Rotterdam (EUR-Fellowship) to MC van Zelm.en_US
dc.identifier.citationArtaç, H., Reisli, İ., Kara, R., Pico-Knijnenburg, I., Adın-Çınar, S., Pekcan, S., Jol-Van der Zijde, C. M., Van Tol, M. J. D., Bakker-Jonges, L. E., Van Dongen, J. J. M., Van der Burg, M., Van Zelm, M. C., (2010). B-Cell Maturation and Antibody Responses in Individuals Carrying a Mutated CD19 Allele. Genes and Immunity, 11(7), 523-530. Doi: 10.1038/gene.2010.22;
dc.identifier.doi10.1038/gene.2010.22en_US
dc.identifier.endpage530en_US
dc.identifier.issn1466-4879en_US
dc.identifier.issue7en_US
dc.identifier.pmid20445561en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage523en_US
dc.identifier.urihttps://dx.doi.org/10.1038/gene.2010.22
dc.identifier.urihttps://hdl.handle.net/20.500.12395/24654
dc.identifier.volume11en_US
dc.identifier.wosWOS:000283247900001en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorArtaç, H.
dc.institutionauthorReisli, İ.
dc.institutionauthorKara, R.
dc.institutionauthorPekcan, S.
dc.language.isoenen_US
dc.publisherNature Publishing Groupen_US
dc.relation.ispartofGenes and Immunityen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.selcuk20240510_oaigen_US
dc.subjectCd19en_US
dc.subjectCd21en_US
dc.subjectB cellen_US
dc.subjectAntibody deficiencyen_US
dc.subjectVaccination responseen_US
dc.subjectHeterozygous mutationen_US
dc.titleB-Cell Maturation and Antibody Responses in Individuals Carrying a Mutated CD19 Alleleen_US
dc.typeArticleen_US

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