Abstract:
:Eleven Norwegian patients (aged 2-33 years, seven males and four females) with Ataxia-telangiectasia (A-T) and their parents were investigated. Five of the patients were homozygous for the same ATM mutation, 3245delATCinsTGAT, a Norwegian founder mutation. They had the lowest IgG2 levels; mean (95% confidence interval) 0.23 (0.05-0.41) g/l versus 0.91 (0.58-1.26) g/l in the other patients (P = 0.002). Among the 11 A-T patients, six had IgG2 deficiency, six had IgA deficiency (three in combination with IgG2 deficiency) and seven had low/undetectable IgE values. All patients had very low levels of antibodies to Streptococcus pneumoniae 0.9 (0.4-1.4) U/ml, while normal levels were found in their parents 11.1 (8.7-13.4) U/ml (P < 0.001). A positive linear relationship between pneumococcal antibodies and IgG2 (r = 0.85, P = 0.001) was found in the patients. Six of 11 had diphtheria antibodies and 7 of 11 tetanus antibodies after childhood vaccinations, while 4 of 7 Hemophilus influenzae type b (Hib) vaccinated patients had protective antibodies. Ten patients had low B cell (CD19+) counts, while six had low T cell (CD3+) counts. Of the T cell subpopulations, 11 had low CD4+ cell counts, six had reduced CD8+ cell counts, and four had an increased portion of double negative (CD3+/CD4-/CD8-) gamma delta T cells. Of the 22 parents (aged 23-64 years) 12 were heterozygous for the ATM founder mutation. Abnormalities in immunoglobulin levels and/or lymphocyte subpopulations were also observed in these carriers, with no correlation to a special ATM genotype.
journal_name
Clin Exp Immunoljournal_title
Clinical and experimental immunologyauthors
Stray-Pedersen A,Jónsson T,Heiberg A,Lindman CR,Widing E,Aaberge IS,Borresen-Dale AL,Abrahamsen TGdoi
10.1111/j.1365-2249.2004.02492.xsubject
Has Abstractpub_date
2004-07-01 00:00:00pages
179-86issue
1eissn
0009-9104issn
1365-2249pii
CEI2492journal_volume
137pub_type
杂志文章abstract::Protein kinases mediate protein phosphorylation, which is a fundamental component of cell signalling, with crucial roles in most signal transduction cascades: from controlling cell growth and proliferation to the initiation and regulation of immunological responses. Aberrant kinase activity is implicated in an increas...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章,评审
doi:10.1111/cei.12248
更新日期:2014-04-01 00:00:00
abstract::Groups of subjects during acute (0-3 days) and convalescent (2-3 weeks) phase of recurrent herpes labialis (RHL), and other subjects seropositive or seronegative for herpes simplex virus type 1 (HSV-1) antibody without any history of RHL, were tested for the appearance of cell-mediated cytotoxic responses by stimulati...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1986-12-01 00:00:00
abstract::Breakpoint cluster region-Abelson murine leukaemia viral oncogene homologue 1 (BCR-ABL1), encoded by the Philadelphia (Ph) chromosome, is the characteristic of chronic myeloid leukaemia (CML) and a subset of acute lymphoblastic leukaemia (ALL). We demonstrated that expression of the Ik6 transcript, which lacked exons ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12812
更新日期:2016-09-01 00:00:00
abstract::We have studied the role of three mouse distinct non-H-2 genes (Bcg, Tbc-1, xid) in several phenomena of antituberculosis immunity and resistance. On the basis of median survival time (MST) of mice following infection with virulent Mycobacterium tuberculosis H37Rv, Bcg gene did not control resistance to the lethal dos...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1996.d01-643.x
更新日期:1996-04-01 00:00:00
abstract::Thymic stromal lymphopoietin (TSLP) is known for its capacity to induce CD11c(+) myeloid dendritic cells to promote T helper type 2 (Th2)-skewed inflammatory responses. Although increased expression of TSLP was reported in the lesional skin of limited numbers of patients with atopic dermatitis (AD), the relationships...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12021
更新日期:2013-03-01 00:00:00
abstract::The synthesis of some class 1 acute-phase proteins (APP), including C-reactive protein (CRP) and serum amyloid A (SAA) protein is completely blocked by the IL-1 receptor antagonist (IL-1Ra), whereas the production of fibrinogen, a class 2 APP, is increased by IL-1Ra in hepatoma cells, but this has never been tested in...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1995.tb03669.x
更新日期:1995-05-01 00:00:00
abstract::We have developed CD4+, tetanus antigen-specific T cell clones that proliferate in the presence of tetanus antigen and autologous irradiated peripheral blood leucocytes (PBL) as antigen-presenting cells (APC). There have been several reports that T cells can present antigen themselves. We have used tetanus antigen-spe...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1990.tb05226.x
更新日期:1990-05-01 00:00:00
abstract::Normal, but not tolerant, adult thymocytes prevented particulate antigen inducing tolerance in differentiating B lymphocytes. This was demonstrated by measuring the emergence of responsive B cells (identified by their allotype) developing from a foetal liver innoculum in irradiated hosts with or without the addition o...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1978-10-01 00:00:00
abstract::Perivascular infiltrates of inflammatory cells are a hallmark of lesional skin in scleroderma. We have explored the potential for scleroderma fibroblasts to modulate mononuclear leucocyte migration across endothelial cell monolayers in tissue culture, and to regulate expression of endothelial cell adhesion molecules. ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1998.00721.x
更新日期:1998-11-01 00:00:00
abstract::There is increasing evidence that the functional state of the immune system at birth is predictive of the kinetics of immune maturation in early infancy. Moreover, this maturation process can have a major impact on early vaccine responses and can be a key determinant of risk for communicable and non-communicable disea...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2010.04137.x
更新日期:2010-04-01 00:00:00
abstract::Autoantibodies to the intermediate filament proteins vimentin and keratin were studied in sera of 50 Caribbean patients with Schistosoma mansoni infection and 50 control subjects. Autoantibodies were detected by indirect immunofluorescence on HEp-2 cells pretreated with colchicine. The incidence of anti-vimentin antib...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1989-08-01 00:00:00
abstract::Individuals with type 1 diabetes (T1D) are at increased risk of coeliac disease (CD), autoimmune thyroiditis and autoimmune gastritis, but the absolute risks are unclear. The aim of this study was to investigate the prevalence of autoantibodies to tissue transglutaminase (TGA), thyroid peroxidase (TPOA) and gastric H+...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.13115
更新日期:2018-06-01 00:00:00
abstract::CD5 is an antigen normally found on T cells and on a minority subpopulation of B cells in fetal spleen and tonsil and on the majority of cells in B-chronic lymphocytic leukaemia. Recent reports described the occurrence of large numbers of CD5-positive B cells in the peripheral blood after bone marrow transplantation (...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1987-06-01 00:00:00
abstract::L-selectin in one of the key members of the selectin family of adhesion molecules and initiates leucocyte attachment to specialized high endothelial venules. The shed form, which retains functional activity, can be detected in biological fluids and is increased in diseases of many kinds. In the present study, we inves...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1996.37744.x
更新日期:1996-05-01 00:00:00
abstract::An injection of human renal tubular fraction, Fx1A, with Freund's complete adjuvant in rats induces Heymann nephritis (HN) with clinical and morphological features similar to rat Fx1A-induced HN. The present study was undertaken to characterize antibody specificities and the corresponding antigen involved in human Fx1...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1987-07-01 00:00:00
abstract::The programmed death-1 (PD-1) receptor ligands, PD-L1 and PD-L2, are co-stimulatory molecules that contribute to the negative regulation of T lymphocyte activation. It is still unclear whether there is correlation between PD-L1 or PD-L2 and tumour-infiltrating dendritic cells (TIDCs) in cutaneous squamous cell carcino...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12921
更新日期:2017-06-01 00:00:00
abstract::Psoriasis (Ps), psoriatic arthritis (PsA) and rheumatoid arthritis (RA) are common diseases dependent on environmental factors that activate the immune system in unknown ways. Mannan is a group of polysaccharides common in the environment; they are potentially pathogenic, because at least some of them induce Ps-, PsA-...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.13268
更新日期:2019-06-01 00:00:00
abstract::Hepatitis C virus (HCV) is an RNA virus which is estimated to persistently infect about 170 million people worldwide. After acute infection, there is an initial period during which long-term outcome is decided. There is strong evidence that the cellular immune responses, involving both CD4+ and CD8+ T lymphocytes, are...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章,评审
doi:10.1046/j.1365-2249.2002.01840.x
更新日期:2002-05-01 00:00:00
abstract::In autoimmune diseases, mitogen-induced IL-2 production in vitro is generally considered to be diminished despite evidence of lymphoid hyperactivity in vivo. HgCl2 is known to cause T-dependent polyclonal B cell activation in Brown-Norway (BN) rats, resulting in autoimmune disease. We show here that the IL-2 producing...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1988-09-01 00:00:00
abstract::Antibody response and antigen specific suppression to bovine serum albumin (BSA) in uremic rats have been investigated. Primary immune response was found to be significantly reduced independent of the route of administration or adjuvant while secondary response was affected only in animals in which a weak adjuvant suc...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-04-01 00:00:00
abstract::The F1 hybrid mouse strain, from B10Q and DBA/1 parentals (the QD strain), is highly susceptible to induction of type II collagen-induced arthritis, an experimental model for rheumatoid arthritis. Males are more susceptible than females. Oophorectomy enhances susceptibility to arthritis and treatment with physiologica...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1992.tb06978.x
更新日期:1992-09-01 00:00:00
abstract::Antibody reactivity against the 'mitochondrial M2 antigen' was determined in sera from 10 patients with primary biliary cirrhosis (PBC), using Western blotting after SDS-PAGE separation of rat liver mitochondria (RLM) and plasma membrane proteins. The molecular weights of the major M2 antigens in rat liver mitochondri...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1991.tb05652.x
更新日期:1991-03-01 00:00:00
abstract::Bone marrow (BM) lymphocytes were obtained by sucrose density gradient centrifugation of the nucleated cells from adult human BM. BM was obtained from rib sections removed routinely during thoracotomy from thirteen patients with a localized lung tumour and from two other patients without tumour (mean age 47 years). Th...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1979-10-01 00:00:00
abstract::The CXC chemokines, monokine induced by interferon (IFN)-gamma (MIG) (CXCL9), IFN-gamma-induced protein 10 (IP-10) (CXCL10) and IFN-inducible T cell alpha chemoattractant (I-TAC) (CXCL11), are known to attract CXCR3- (CXCR3A and CXCR3B) T lymphocytes. We investigated MIG, IP-10 and I-TAC mRNAs expression by semi-quant...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12032
更新日期:2013-04-01 00:00:00
abstract::There is a link between increased allergy and a reduction of some infections in western countries. Epidemiological data also show that respiratory allergy is less frequent in people exposed to orofaecal and foodborne microbes such as Toxoplasma gondii. Infection with T. gondii induces a strong cell-mediated immunity w...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2008.03813.x
更新日期:2009-02-01 00:00:00
abstract::Serum and salivary responses of 95 infants to either a standard (4 x 10(4) plaque-forming units (PFU), 47 neonates) or a high dose (4 x 10(5) PFU, 48 neonates) of tetravalent reassortant rhesus rotavirus vaccine (administered at 2 days and at 6 weeks of age) were evaluated in a double-blind clinical trial. Serum and s...
journal_title:Clinical and experimental immunology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1365-2249.1993.tb03379.x
更新日期:1993-05-01 00:00:00
abstract::Rheumatoid arthritis (RA) is an autoimmune disease characterized by chronic inflammation, local and systemic bone loss and a lack of compensatory bone repair. Fibroblast-like synoviocytes (FLS) are the most abundant cells of the stroma and a key population in autoimmune diseases such as RA. An increasing body of evide...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章,评审
doi:10.1111/cei.12979
更新日期:2017-07-01 00:00:00
abstract::T cells implicated in chronic inflammatory diseases such as RA respond weakly when stimulated in vitro with mitogen or antigen. The mechanism behind this hyporesponsiveness is unclear, but a depressed expression of the T cell receptor (TCR)-associated CD3zeta chain has been suggested. In the present work we describe a...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.2000.01180.x
更新日期:2000-04-01 00:00:00
abstract::Expression of the inducible form of nitric oxide synthase (iNOS) has been found to be up-regulated in cytokine-stimulated mesangial cells (MC) and in experimental glomerulonephritis. Since direct toxicity of nitric oxide (NO) has been implicated in damage of bacteria, neoplastic and intact pancreatic cells, we investi...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1997.d01-906.x
更新日期:1997-01-01 00:00:00
abstract::The anti-EBV T cell response was studied in a severe combined immunodeficiency patient (PS) who received two transplants of fetal liver cells. His peripheral blood mononuclear cells (PBMC) were incubated with EBV and cultured during 15 days. Eleven colonies were derived from the T lymphocytes causing the regression of...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1993-12-01 00:00:00