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Article: Epstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and Wiskott-Aldrich Syndrome: responses to B cell growth and differentiation factors

TitleEpstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and Wiskott-Aldrich Syndrome: responses to B cell growth and differentiation factors
Authors
Issue Date1989
PublisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/CEI
Citation
Clinical And Experimental Immunology, 1989, v. 75 n. 2, p. 190-195 How to Cite?
AbstractEpstein-Barr-virus-transformed B lymphoblastoid cell lines (EBV-transformed LCL) from three patients with X-linked agammaglobulinaemia (XLA), six patients with Wiskott-Aldrich Syndrome (WAS), and seven normal donors, were tested for growth and differentiation in response to human recombinant IL-4, a commercially available, low molecular weight B cell growth factor (BCGF(low)), and B cell differentiation factor (BCDF) secreted by the T24 cell line, now known to be IL-6. Proliferation (3H-TdR uptake) by EBV-transformed LCL from both XLA and WAS patients in response to BCGF(low) was similar to that obtained with the normal cell lines. In addition, three normal and three WAS, but none of the XLA EBV-transformed LCL, proliferated a little in response to IL-4. All the normal B cell lines secreted IgM, and six out of the seven secreted IgG in response to BCGF(low) and BCDF. A similar pattern of response was obtained with the WAS EBV-transformed LCL (6/6 secreted IgM and 4/6 secreted IgG). Several of the normal and WAS EBV-transformed LCL also secreted IgM and IgG in response to IL-4. In contrast, the lines from the XLA patients were abnormal. One secreted large amounts of IgM and two secreted small amounts, but none of the XLA lines secreted IgG constitutively or in response to any of the factors (IL-4, BCGF(low), BCDF). The lack of detectable IgG secretion by the XLA lines was probably due to an absence of precommitted IgG B cell precursors transformed by EBV rather than an intrinsic inability to respond to BCGF and BCDF. All of the lines, including those derived from XLA patients, were shown to secrete B cell growth and differentiation factors detected on indicator B cell lines. These results suggest that the abnormal X-linked genes responsible for XLA and WAS do not interfere with B cell responses to B cell growth and differentiation factors.
Persistent Identifierhttp://hdl.handle.net/10722/170238
ISSN
2015 Impact Factor: 3.148
2015 SCImago Journal Rankings: 1.369

 

DC FieldValueLanguage
dc.contributor.authorLau, YLen_US
dc.contributor.authorShields, JGen_US
dc.contributor.authorLevinsky, RJen_US
dc.contributor.authorCallard, REen_US
dc.date.accessioned2012-10-30T06:06:54Z-
dc.date.available2012-10-30T06:06:54Z-
dc.date.issued1989en_US
dc.identifier.citationClinical And Experimental Immunology, 1989, v. 75 n. 2, p. 190-195en_US
dc.identifier.issn0009-9104en_US
dc.identifier.urihttp://hdl.handle.net/10722/170238-
dc.description.abstractEpstein-Barr-virus-transformed B lymphoblastoid cell lines (EBV-transformed LCL) from three patients with X-linked agammaglobulinaemia (XLA), six patients with Wiskott-Aldrich Syndrome (WAS), and seven normal donors, were tested for growth and differentiation in response to human recombinant IL-4, a commercially available, low molecular weight B cell growth factor (BCGF(low)), and B cell differentiation factor (BCDF) secreted by the T24 cell line, now known to be IL-6. Proliferation (3H-TdR uptake) by EBV-transformed LCL from both XLA and WAS patients in response to BCGF(low) was similar to that obtained with the normal cell lines. In addition, three normal and three WAS, but none of the XLA EBV-transformed LCL, proliferated a little in response to IL-4. All the normal B cell lines secreted IgM, and six out of the seven secreted IgG in response to BCGF(low) and BCDF. A similar pattern of response was obtained with the WAS EBV-transformed LCL (6/6 secreted IgM and 4/6 secreted IgG). Several of the normal and WAS EBV-transformed LCL also secreted IgM and IgG in response to IL-4. In contrast, the lines from the XLA patients were abnormal. One secreted large amounts of IgM and two secreted small amounts, but none of the XLA lines secreted IgG constitutively or in response to any of the factors (IL-4, BCGF(low), BCDF). The lack of detectable IgG secretion by the XLA lines was probably due to an absence of precommitted IgG B cell precursors transformed by EBV rather than an intrinsic inability to respond to BCGF and BCDF. All of the lines, including those derived from XLA patients, were shown to secrete B cell growth and differentiation factors detected on indicator B cell lines. These results suggest that the abnormal X-linked genes responsible for XLA and WAS do not interfere with B cell responses to B cell growth and differentiation factors.en_US
dc.languageengen_US
dc.publisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/CEIen_US
dc.relation.ispartofClinical and Experimental Immunologyen_US
dc.subject.meshAgammaglobulinemia - Genetics - Immunologyen_US
dc.subject.meshB-Lymphocytes - Immunologyen_US
dc.subject.meshCell Divisionen_US
dc.subject.meshCell Line, Transformeden_US
dc.subject.meshCell Transformation, Viralen_US
dc.subject.meshGenetic Linkageen_US
dc.subject.meshHerpesvirus 4, Humanen_US
dc.subject.meshHumansen_US
dc.subject.meshInterleukin-4en_US
dc.subject.meshInterleukin-6en_US
dc.subject.meshInterleukins - Biosynthesis - Pharmacologyen_US
dc.subject.meshWiskott-Aldrich Syndrome - Immunologyen_US
dc.subject.meshX Chromosomeen_US
dc.titleEpstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and Wiskott-Aldrich Syndrome: responses to B cell growth and differentiation factorsen_US
dc.typeArticleen_US
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_US
dc.identifier.authorityLau, YL=rp00361en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid2539277-
dc.identifier.scopuseid_2-s2.0-0024517244en_US
dc.identifier.volume75en_US
dc.identifier.issue2en_US
dc.identifier.spage190en_US
dc.identifier.epage195en_US
dc.publisher.placeUnited Kingdomen_US
dc.identifier.scopusauthoridLau, YL=7201403380en_US
dc.identifier.scopusauthoridShields, JG=7402027856en_US
dc.identifier.scopusauthoridLevinsky, RJ=7006539367en_US
dc.identifier.scopusauthoridCallard, RE=7005116699en_US

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