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 |
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container_end_page | 1980 |
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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 |
format | Article |
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[...]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).</description><identifier>ISSN: 0091-6749</identifier><identifier>EISSN: 1097-6825</identifier><identifier>DOI: 10.1016/j.jaci.2021.01.027</identifier><identifier>PMID: 33571536</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>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</subject><ispartof>Journal of allergy and clinical immunology, 2021-05, Vol.147 (5), p.1977-1980</ispartof><rights>2021 The Authors</rights><rights>2021. 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[...]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).</description><subject>Agammaglobulinemia</subject><subject>B-cell receptor</subject><subject>BLyS protein</subject><subject>CD22 antigen</subject><subject>CD27 antigen</subject><subject>CD86 antigen</subject><subject>Cell differentiation</subject><subject>Cell lines</subject><subject>Immunoglobulin A</subject><subject>Immunoglobulin G</subject><subject>Immunoglobulins</subject><subject>Lymphocytes B</subject><subject>Lymphoma</subject><subject>Memory cells</subject><subject>Mutation</subject><subject>Nervous system</subject><subject>Oct-1 protein</subject><subject>Phosphorylation</subject><subject>Syk protein</subject><subject>Transcription factors</subject><issn>0091-6749</issn><issn>1097-6825</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><recordid>eNp9kE1LAzEQhoMotlb_gAcJeN41k89d8FKLVqHgRc8hm01r1v2o2V3Ff29Kq0dhYBh433dmHoQugaRAQN5UaWWsTymhkJJYVB2hKZBcJTKj4hhNCckhkYrnE3TW9xWJM8vyUzRhTCgQTE7Rcr4xTWM2dVeMtW9d4w3-8sMbbrvQmBrfJdbVNW7HpnChx77FBm_N4F074NrYd99u8F1XwDk6WZu6dxeHPkOvD_cvi8dk9bx8WsxXiWW5GBJheMY5LwysSy5lTsuiEByEYhwAmONgVW5KkDTeSkuZOSu5IFxkTDJFLJuh633uNnQfo-sHXXVjaONKTQWlRIDMVFTRvcqGru-DW-tt8I0J3xqI3rHTld6x0zt2msSiO9PVIXosGlf-WX5hRcHtXuDig5_eBd3bCMK60gdnB112_r_8HyQzfNY</recordid><startdate>202105</startdate><enddate>202105</enddate><creator>Kury, Patrick</creator><creator>Staniek, Julian</creator><creator>Wegehaupt, Oliver</creator><creator>Janowska, Iga</creator><creator>Eckenweiler, Matthias</creator><creator>Korinthenberg, Rudolf</creator><creator>Japaridze, Natia</creator><creator>Pendziwiat, Manuela</creator><creator>Helbig, Ingo</creator><creator>Verhoeyen, Els</creator><creator>Jung, Johannes</creator><creator>Garcia de Oteyza, Andres Caballero</creator><creator>Proietti, Michele</creator><creator>Phirtskhalaishvili, Tamar</creator><creator>Rtskhiladze, Irakli</creator><creator>Nielsen, Peter J.</creator><creator>Ehl, Stephan</creator><creator>Speckmann, Carsten</creator><creator>Rizzi, Marta</creator><general>Elsevier Inc</general><general>Elsevier Limited</general><scope>6I.</scope><scope>AAFTH</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7SS</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><orcidid>https://orcid.org/0000-0002-5153-6089</orcidid></search><sort><creationdate>202105</creationdate><title>Agammaglobulinemia with normal B-cell numbers in a patient lacking Bob1</title><author>Kury, Patrick ; 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[...]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).</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>33571536</pmid><doi>10.1016/j.jaci.2021.01.027</doi><tpages>4</tpages><orcidid>https://orcid.org/0000-0002-5153-6089</orcidid><oa>free_for_read</oa></addata></record> |
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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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