Agammaglobulinemia with normal B-cell numbers in a patient lacking Bob1

In B cells, Bob1 is a transcriptional coactivator that confers octamer-dependent specificity to the transcription factors Oct-1 and Oct-2.1 P-FR1 had normal B-cell numbers but disturbed B-cell differentiation, with decreased class-switched memory cells and a relative increase in atypical-memory cell...

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Veröffentlicht in:Journal of allergy and clinical immunology 2021-05, Vol.147 (5), p.1977-1980
Hauptverfasser: Kury, Patrick, Staniek, Julian, Wegehaupt, Oliver, Janowska, Iga, Eckenweiler, Matthias, Korinthenberg, Rudolf, Japaridze, Natia, Pendziwiat, Manuela, Helbig, Ingo, Verhoeyen, Els, Jung, Johannes, Garcia de Oteyza, Andres Caballero, Proietti, Michele, Phirtskhalaishvili, Tamar, Rtskhiladze, Irakli, Nielsen, Peter J., Ehl, Stephan, Speckmann, Carsten, Rizzi, Marta
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container_end_page 1980
container_issue 5
container_start_page 1977
container_title Journal of allergy and clinical immunology
container_volume 147
creator Kury, Patrick
Staniek, Julian
Wegehaupt, Oliver
Janowska, Iga
Eckenweiler, Matthias
Korinthenberg, Rudolf
Japaridze, Natia
Pendziwiat, Manuela
Helbig, Ingo
Verhoeyen, Els
Jung, Johannes
Garcia de Oteyza, Andres Caballero
Proietti, Michele
Phirtskhalaishvili, Tamar
Rtskhiladze, Irakli
Nielsen, Peter J.
Ehl, Stephan
Speckmann, Carsten
Rizzi, Marta
description In B cells, Bob1 is a transcriptional coactivator that confers octamer-dependent specificity to the transcription factors Oct-1 and Oct-2.1 P-FR1 had normal B-cell numbers but disturbed B-cell differentiation, with decreased class-switched memory cells and a relative increase in atypical-memory cells (IgG+ or IgA+ CD27– [Fig 1, D and E]). [...]Bob1 can directly regulate expression of B-cell receptor (BCR) signaling molecules by interacting with octamer motifs in their promoter regions, as described for CD79β.2 Low CD79β may contribute to low BCR and CD79α expression, as shown in B-cell lines.3 Additionally, interaction with Bob1 has been reported to control the stability of Syk.4 Also, BAFF-R expression and CD22 expression were reduced in P-FR1 B cells. [...]activated P-FR1–derived B cells failed to upregulate CD86 (see Fig E3, A in the Online Repository at www.jacionline.org).
doi_str_mv 10.1016/j.jaci.2021.01.027
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subjects Agammaglobulinemia
B-cell receptor
BLyS protein
CD22 antigen
CD27 antigen
CD86 antigen
Cell differentiation
Cell lines
Immunoglobulin A
Immunoglobulin G
Immunoglobulins
Lymphocytes B
Lymphoma
Memory cells
Mutation
Nervous system
Oct-1 protein
Phosphorylation
Syk protein
Transcription factors
title Agammaglobulinemia with normal B-cell numbers in a patient lacking Bob1
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