Abstract:
:Mutations in the Bruton's tyrosine kinase (BTK) gene result in XLA. Despite the large numbers of BTK mutations reported, no correlation can be made between the clinical phenotype and the gene defects. Analysis of Btk protein expression and activity in individuals with XLA was performed to characterize the relationship between a particular mutation, the resultant Btk protein and the clinical phenotype. In most patients studied, including those with atypical phenotypes, there was complete absence of protein expression and activity. Furthermore, in two undiagnosed individuals with a clinical phenotype suggestive of XLA, lack of protein expression was used to confirm an abnormality in Btk. These results underline the importance of protein analysis prior to speculating on protein structure and function based on the gene mutation. Lack of Btk expression in atypical phenotypes suggests that there is redundancy in B lymphocyte signalling such that alternative signalling molecules, or mechanisms, can compensate for the lack of Btk. We also suggest that analysis of Btk expression can be used as an indicator of XLA. These rapid assays may be used to screen a wider spectrum of individuals with humoral immunodeficiency in order to characterize fully the extent of Btk deficiency.
journal_name
Clin Exp Immunoljournal_title
Clinical and experimental immunologyauthors
Gaspar HB,Lester T,Levinsky RJ,Kinnon Csubject
Has Abstractpub_date
1998-02-01 00:00:00pages
334-8issue
2eissn
0009-9104issn
1365-2249journal_volume
111pub_type
杂志文章abstract::Anticardiolipin antibodies (ACA) belong to a heterogeneous group of antibodies directed against negatively charged phospholipids. In patients with rheumatic disorders, their presence has been correlated to the occurrence of thromboembolic complications, thrombocytopenia, abortions and other disease manifestations. Sev...
journal_title:Clinical and experimental immunology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1365-2249.1994.tb05498.x
更新日期:1994-12-01 00:00:00
abstract::Autoantibodies directed against the Sm proteins of the spliceosome complex are found in approximately 25% of systemic lupus erythematosus (SLE) patients sera. To determine which regions of the Sm D polypeptide are involved in the lupus autoimmune response, binding to overlapping octapeptides of Sm D has been evaluated...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1994.tb05507.x
更新日期:1994-12-01 00:00:00
abstract::Reliable risk assessment for biotherapeutics requires accurate evaluation of risk factors associated with immunogenicity. Immunogenicity risk assessment tools were developed and applied to investigate the immunogenicity of a fully human therapeutic monoclonal antibody, ATR-107 [anti-interleukin (IL)-21 receptor] that ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12711
更新日期:2016-01-01 00:00:00
abstract::Mucous membrane pemphigoid (MMP) (also known as cicatricial pemphigoid) is a rare autoimmune mucocutaneous blistering disease that affects mucous membranes derived from stratified squamous epithelium and the skin. A subset of MMP affects only the oral cavity and is referred to as the oral pemphigoid (OP). MMP and OP a...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2006.03103.x
更新日期:2006-07-01 00:00:00
abstract::Double-labelling immunofluorescence analysis within the CD4+ cell subset was carried out in 27 bronchoalveolar lavage fluids and 11 peripheral blood samples of sarcoidosis patients with anti-TQ1, anti-2H4 and anti-4B4 monoclonal antibodies. Helper/inducer CD4+TQ1-/4B4+ cells were strongly increased in the lung and sli...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1988-08-01 00:00:00
abstract::Antibody titres to whole ovary, theca cells, granulosa cells and endometrium were determined by passive haemagglutination and immunofluorescence assays in sera and in cervical and vaginal secretions from 13 patients with endometriosis. Antibody titres to endometrium (mean log2 +/- s.e.m., 7.08 +/- 0.80; P less than 0....
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-11-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12369
更新日期:2014-09-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-08-01 00:00:00
abstract::Oral immunization with 25 mg/day of ovalbumin resulted in a number of changes in non-specific immune responsiveness, including increases in antibody produced to T-dependent but not T-independent antigens and the abrogation of tolerance induced by both oral and intravenous routes. The effect was transient, sex- and str...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1983-05-01 00:00:00
abstract::ABA-1 is a major allergen of nematode parasites of the genus Ascaris which includes the large roundworms of humans and pigs, A. lumbricoides and A. suum, respectively. The allergen was purified from A. suum by immunoaffinity chromatography for immunochemical examination. The IgE antibody repertoire is under MHC contro...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1993.tb05958.x
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1993.tb03442.x
更新日期:1993-11-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.13087
更新日期:2018-04-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章,评审
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更新日期:2009-12-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2005.02802.x
更新日期:2005-06-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-05-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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更新日期:2007-11-01 00:00:00
abstract::In addition to its activity as a metabolic hormone and a regulator of somatic growth, insulin-like growth factor-I (IGF-I) has cytokine-like activities on lymphoid cells. A 14-day infusion of recombinant human (rh)IGF-I increased lymphocyte numbers in all the peripheral lymphoid organs examined. This increase was appa...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1994.tb06534.x
更新日期:1994-02-01 00:00:00
abstract::Immunopathological mechanisms are speculated to underlie haemorrhagic fever with renal syndrome (HFRS) caused by Hantaviruses. CD4+CD25+ T regulatory cells (T(regs)), a subset of CD4+ T cells, expressed high levels of CD25 and the forkhead box transcription factor P3 (FoxP3), plays an important role in the down-regula...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2008.03858.x
更新日期:2009-04-01 00:00:00
abstract::Presence of antibodies directed against replication protein A (RPA), a DNA binding protein complex composed of three subunits (RPA-70, RPA-32 and RPA-14) was investigated among patients with SLE and other autoimmune diseases using immunoblot analysis to RPA-70 and RPA-32 recombinant proteins. Anti-RPA antibodies were ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1985-12-01 00:00:00
abstract::The lymphocyte activating properties of a membrane proteoglycan (MPG) extracted from a mutant non-encapsulated strain of Klebsiella pneumoniae (Kp) (biotype a I-145) were investigated. Kp MPG induced a strong proliferative response of BALB/c spleen cells and Peyer's patches cells. Thymidine incorporation was dose-rela...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1987-10-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1993-12-01 00:00:00
abstract::We recently separated a PG1828-encoded triacylated lipoprotein (Pg-LP), composed of two palmitoyl and one pentadecanoyl groups at the N-terminal of glycerocysteine from Porphyromonas gingivalis, a periodontopathic bacteria, and found that Pg-LP exhibited definite biological activities through Toll-like receptor (TLR) ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1993.tb07972.x
更新日期:1993-08-01 00:00:00
abstract::A preparation of the putative antibody in HN (Heymann's nephritis) lacking preformed immune complexes, obtained from the acid eluates of kidneys with HN, purified through ion exchange and gel-filtration chromatography, characterized by immunoelectrophoresis, sucrose density-gradient ultracentrifugation, 125I-Clq assay...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-02-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
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更新日期:1990-04-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2005.02949.x
更新日期:2005-12-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2011.04431.x
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