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    2465 results match your criteria Immunoglobulin M Deficiency

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    The decreased growth performance and impaired immune function and structural integrity by dietary iron deficiency or excess are associated with TOR, NF-κB, p38MAPK, Nrf2 and MLCK signaling in head kidney, spleen and skin of grass carp (Ctenopharyngodon idella).
    Fish Shellfish Immunol 2017 Jun 18;65:145-168. Epub 2017 Apr 18.
    Animal Nutrition Institute, Sichuan Agricultural University, Chengdu 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Chengdu 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Chengdu 611130, China. Electronic address:
    This study was conducted to investigate the effects of dietary iron on the growth, and immune function and structural integrity in head kidney, spleen and skin as well as the underlying signaling of young grass carp (Ctenopharyngodon idella). Total 630 grass carp (242.32 ± 0. Read More

    Changes in the Level of Immunoglobulins and CD4/CD8 Ratio in Young and Aged Mice with Estradiol Deficiency.
    Immunol Invest 2017 Apr 7;46(3):305-313. Epub 2017 Feb 7.
    a Department of Obstetrics and Gynecology , Jinhua Municipal Central Hospital , Zhejiang Province , People's Republic of China.
    Studies demonstrated that deficiency in 17β-estradiol (E2) in postmenopausal women influences their immune system. However, few studies have reported alterations in immunologic presentation during nonnatural menopause in young females. Here we compared the differences in immune response between young C57BL/6N mice with surgical or medical variectomy and aged C57BL/6N mice with the common feature of E2 deficiency following Con A stimulation. Read More

    Effect of dietary phosphorus deficiency on the growth, immune function and structural integrity of head kidney, spleen and skin in young grass carp (Ctenopharyngodon idella).
    Fish Shellfish Immunol 2017 Apr 10;63:103-126. Epub 2017 Feb 10.
    Animal Nutrition Institute, Sichuan Agricultural University, Sichuan, Chengdu 611130, China; Fish Nutrition and Safety Production University Key Laboratory of Sichuan Province, Sichuan Agricultural University, Sichuan, Chengdu 611130, China; Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Sichuan, Chengdu 611130, China. Electronic address:
    This study evaluates the effects of dietary phosphorus on the growth, immune function and structural integrity (head kidney, spleen and skin) of young grass carp (Ctenopharyngodon idella) that were fed graded levels of available phosphorus (0.95-8.75 g/kg diet). Read More

    The IgM receptor FcμR limits tonic BCR signaling by regulating expression of the IgM BCR.
    Nat Immunol 2017 Mar 30;18(3):321-333. Epub 2017 Jan 30.
    Center for Comparative Medicine, University of California, Davis, Davis, California, USA.
    The FcμR receptor for the crystallizable fragment (Fc) of immunoglobulin M (IgM) can function as a cell-surface receptor for secreted IgM on a variety of cell types. We found here that FcμR was also expressed in the trans-Golgi network of developing B cells, where it constrained transport of the IgM-isotype BCR (IgM-BCR) but not of the IgD-isotype BCR (IgD-BCR). In the absence of FcμR, the surface expression of IgM-BCR was increased, which resulted in enhanced tonic BCR signaling. Read More

    Increased Plasma IgE Accelerate Atherosclerosis in Secreted IgM Deficiency.
    Circ Res 2017 Jan 30;120(1):78-84. Epub 2016 Nov 30.
    From the CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria (D.T., M.O.-K., L.G., K.H., C.J.B.); Department of Laboratory Medicine, Medical University of Vienna, Austria (D.T., M.O.-K., L.G., T.P., K.H., C.J.B.); Division of Biopharmaceutics, LACDR Leiden University, The Netherlands (I.B., J.K.); Department of Microbiology and Immunology, Virginia Commonwealth University, Richmond (D.H.C.); and Division of Cardiovascular Medicine, University of Cambridge, United Kingdom (Z.M.).
    Rationale: Deficiency of secreted IgM (sIgM(-/-)) accelerates atherosclerosis in Ldlr(-/-)mice. Several atheroprotective effects of increased levels of IgM antibodies have been suggested, including preventing inflammation induced by oxidized low-density lipoprotein and promoting apoptotic cell clearance. However, the mechanisms by which the lack of sIgM promotes lesion formation remain unknown. Read More

    LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells.
    Acta Med Iran 2016 Oct;54(10):620-623
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. AND Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. AND Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA.
    LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. Read More

    Transgenic mouse model of IgM(+) lymphoproliferative disease mimicking Waldenström macroglobulinemia.
    Blood Cancer J 2016 Nov 4;6(11):e488. Epub 2016 Nov 4.
    Department of Pathology, Iowa Institute of Human Genetics, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA, USA.
    Waldenström macroglobulinemia (WM) is a low-grade incurable immunoglobulin M(+) (IgM(+)) lymphoplasmacytic lymphoma for which a genetically engineered mouse model of de novo tumor development is lacking. On the basis of evidence that the pro-inflammatory cytokine, interleukin 6 (IL6), and the survival-enhancing oncoprotein, B cell leukemia 2 (BCL2), have critical roles in the natural history of WM, we hypothesized that the enforced expression of IL6 and BCL2 in mice unable to perform immunoglobulin class switch recombination may result in a lymphoproliferative disease that mimics WM. To evaluate this possibility, we generated compound transgenic BALB/c mice that harbored the human BCL2 and IL6 transgenes, EμSV-BCL2-22 and H2-L(d)-hIL6, on the genetic background of activation-induced cytidine deaminase (AID) deficiency. Read More

    Novel AICDA mutation in a case of autosomal recessive hyper-IgM syndrome, growth hormone deficiency and autoimmunity.
    Allergol Immunopathol (Madr) 2017 Jan - Feb;45(1):82-86. Epub 2016 Oct 24.
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Sheffield, UK. Electronic address:
    Background: The Hyper-immunoglobulin M syndromes (HIGM) are a heterogeneous group of genetic disorders, which have been rarely reported to be associated with growth hormone deficiency (GHD).

    Methods And Results: A nine-year-old girl with recurrent urinary tract infections, diarrhoea, sinopulmonary infections, and failure to thrive since the age of six months had normal CD3+, CD4+, CD8+T lymphocytes, and CD19+B lymphocytes and natural killer (NK) cells, but extremely elevated IgM and significantly decreased IgG and IgA. In view of the patient's short stature, growth hormone evaluation was carried out and growth hormone deficiency established. Read More

    Symptomatic Primary Selective IgM Immunodeficiency - B Lymphoid Cell Defect in Adult Man with Secondary HLH Syndrome.
    J Assoc Physicians India 2016 Jul;64(7):91-93
    Clinical Director, Narayana Multispecialty Hospital, Jaipur, Rajasthan.
    Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. Read More

    FcμR in human B cell subsets in primary selective IgM deficiency, and regulation of FcμR and production of natural IgM antibodies by IGIV.
    Hum Immunol 2016 Dec 14;77(12):1194-1201. Epub 2016 Oct 14.
    Division of Basic and Clinical Immunology, University of California at Irvine, Irvine, CA, USA.
    IgMFcR (FcμR) are expressed on B cell and B cell subsets. Mice deficient in secreted IgM and FcμR share properties of impaired specific antibody response and autoimmunity with patient with selective IgM deficiency (SIGMD). Intravenous immunoglobulin (IGIV) regulates immune response, including modulation of IgGFc receptors. Read More

    Symptomatic Primary Selective Immunoglobulin M Deficiency with Nonprotective Pneumococcal Titers Responsive to Subcutaneous Immunoglobulin Treatment.
    Int Arch Allergy Immunol 2016 10;170(2):138-40. Epub 2016 Aug 10.
    Division of Allergy and Immunology, Department of Internal Medicine, University of South Florida, Morsani College of Medicine, James A. Haley Veterans' Affairs Hospital, Tampa, Fla., USA.
    Selective immunoglobulin M deficiency (SIgMD) is a rare disorder with varying clinical features. The prevalence of SIgMD is 0.03-3%. Read More

    Venous Thromboembolism After Knee Arthroscopy in Undiagnosed Familial Thrombophilia.
    Orthopedics 2016 Nov 27;39(6):e1052-e1057. Epub 2016 Jul 27.
    Venous thromboembolism is uncommon after knee arthroscopy, and there are no guidelines for thromboprophylaxis in elective routine knee arthroscopy. Preoperative evaluation of common thrombophilias should provide guidance for postarthroscopy thromboprophylaxis in otherwise healthy patients who are at high risk for venous thromboembolism. This study assessed 10 patients with venous thromboembolism after total hip or knee arthroplasty. Read More

    [Seroprevalence of human parvovirus B19 in children with fever and rash in the North of Tunisia].
    Bull Soc Pathol Exot 2016 Aug 7;109(3):165-71. Epub 2016 Jul 7.
    Unité de recherche UR12ES01, faculté de médecine de Tunis, université de Tunis El-Manar, Tunis, Tunisie.
    The aim of the study is to evaluate the prevalence of specific antibodies anti-human parvovirus B19 (PVB19) immunoglobulin M (IgM) and IgG in children with fever and rash. This study involved 257 children aged from 7 months to 15 years with febrile rash unrelated to measles and rubella (seronegative for IgM). The sera were examined by immunoenzymatic assay. Read More

    The Borrelia burgdorferi CheY3 response regulator is essential for chemotaxis and completion of its natural infection cycle.
    Cell Microbiol 2016 Dec 11;18(12):1782-1799. Epub 2016 Jul 11.
    Department of Microbiology and Immunology, Brody School of Medicine, East Carolina University, Greenville, NC, USA.
    Borrelia burgdorferi possesses a sophisticated and complex chemotaxis system, but how the organism utilizes this system in its natural enzootic life cycle is poorly understood. Of the three CheY chemotaxis response regulators in B. burgdorferi, we found that only deletion of cheY3 resulted in an altered motility and significantly reduced chemotaxis phenotype. Read More

    Clinical Associations of Biallelic and Monoallelic TNFRSF13B Variants in Italian Primary Antibody Deficiency Syndromes.
    J Immunol Res 2016 30;2016:8390356. Epub 2016 Mar 30.
    Department of Medical Genetics, Policlinico S. Orsola-Malpighi, Medical University of Bologna, Via Massarenti 9, 40138 Bologna, Italy.
    We assessed the prevalence of TNFRSF13B mutations and the clinical correlates in an Italian cohort of 189 CVID, 67 IgAD patients, and 330 healthy controls to substantiate the role of TACI genetic testing in diagnostic workup. We found that 11% of CVID and 13% of IgAD carried at least one mutated TNFRSF13B allele. Seven per cent of CVID had monoallelic-mutations and 4% had biallelic-mutations. Read More

    Selective Subnormal IgG1 in 54 Adult Index Patients with Frequent or Severe Bacterial Respiratory Tract Infections.
    J Immunol Res 2016 31;2016:1405950. Epub 2016 Mar 31.
    Southern Iron Disorders Center, Birmingham, AL 35209, USA; Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
    We characterized 54 adult index patients with reports of frequent or severe bacterial respiratory tract infections at diagnosis of selective subnormal IgG1. Mean age was 50 ± 13 (SD) y; 87.0% were women. Read More

    Sialic Acid-Binding Immunoglobulin-like Lectin G Promotes Atherosclerosis and Liver Inflammation by Suppressing the Protective Functions of B-1 Cells.
    Cell Rep 2016 Mar 3;14(10):2348-61. Epub 2016 Mar 3.
    CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, 1090 Vienna, Austria; Department of Laboratory Medicine, Medical University of Vienna, 1090 Vienna, Austria. Electronic address:
    Atherosclerosis is initiated and sustained by hypercholesterolemia, which results in the generation of oxidized LDL (OxLDL) and other metabolic byproducts that trigger inflammation. Specific immune responses have been shown to modulate the inflammatory response during atherogenesis. The sialic acid-binding immunoglobulin-like lectin G (Siglec-G) is a negative regulator of the functions of several immune cells, including myeloid cells and B-1 cells. Read More

    Selective immunoglobulin M deficiency in an adult with miliary tuberculosis: A clinically interesting coexistence. A case report and review of the literature.
    Int J Mycobacteriol 2016 Mar 7;5(1):106-10. Epub 2015 Dec 7.
    Department of Internal Medicine, Hematology Division, Faculty of Medicine, Sohag University Hospital, Sohag, Egypt. Electronic address:
    Selective immunoglobulin M (SIgM) deficiency is a rare form of dysgammaglobulinemia. Here we are reporting a 31year old man with multiple cervical and testicular abscesses who was investigated and found to have miliary tuberculosis (MTB) with primary SIgM deficiency (Serum IgM: 17.4mg/dL) and was treated aggressively with anti-tuberculous treatment. Read More

    Cohort of Iranian Patients with Congenital Agammaglobulinemia: Mutation Analysis and Novel Gene Defects.
    Expert Rev Clin Immunol 2016 24;12(4):479-86. Epub 2016 Feb 24.
    a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
    Objectives: Impairment in early B-cell development can cause a predominantly antibody deficiency with severe depletion of peripheral B-cells. Mutations in the gene encoding for Bruton's-tyrosine-kinase (BTK) and the components of the pre-B-cell receptor complex or downstream signaling molecules have been related to this defect in patients with agammaglobulinemia.

    Methods: Iranian patients with congenital agammaglobulinemia were included and the correlation between disease-causing mutations and parameters such as clinical and immunologic phenotypes were evaluated in available patients. Read More

    Immunogenicity of Renal Microvascular Endothelial Cells From Genetically Modified Pigs.
    Transplantation 2016 Mar;100(3):533-7
    1 Department of Surgery, Indiana University School of Medicine, Indianapolis, IN.2 Department of Surgery, Indiana University Health, Indianapolis, IN.3 IU Health Transplant Institute, Indianapolis, IN.
    Background: Disrupting the porcine GGTA1 and CMAH genes [double knockout (DKO)] that produce the gal-α(1,3)-gal and N-glycolylneuraminic acid xenoantigens reduces human antibody binding to porcine peripheral blood mononuclear cells. It is important to examine rejection pathways at an organ-specific level. The object of this study is to evaluate the human preformed antibody reactivity against DKO renal microvascular endothelial cells (RMEC) in vitro. Read More

    Aberrant levels of natural IgM antibodies in osteoarthritis and rheumatoid arthritis patients in comparison to healthy controls.
    Immunol Lett 2016 Feb 29;170:27-36. Epub 2015 Dec 29.
    Perinatal Research, Royal North Shore Hospital, Sydney, NSW 2065, Australia; Northern Clinical School, University of Sydney, Royal North Shore Hospital, Sydney, NSW 2065, Australia.
    Natural IgM antibodies (nIgM) are polyreactive autoantibodies that have diverse roles in regulating autoimmunity, systemic inflammation and removal of oxidized low-density lipoproteins (oxLDL). We hypothesized that aberrant states of nIgM may exist in persons with osteoarthritis (OA) and rheumatoid arthritis (RA). Herein, we characterized and compared the levels of nIgM specific for phosphorylcholine (anti-PC), double-stranded DNA (anti-dsDNA), and galactosyl (anti-Gal) in persons with OA, RA and healthy controls (HC). Read More

    A role of oestrogen in aggravating SLE-like syndrome in C4-deficient mice.
    Asian Pac J Allergy Immunol 2015 Dec;33(4):339-48
    Department of Anatomy, Faculty of Science, Mahidol University, Bangkok 10400, Thailand.
    Background: As one of the epigenetic factors, oestrogen is considered to be a predisposing factor that is associated with a susceptibility to autoimmune disease development in women including systemic lupus erythematosus (SLE). Here, we proposed that oestrogen is also imparted in a post-lupus symptomatic enhancement as studied in the C4-deficient (C4-/-) mice model known to develop SLE-like symptoms.

    Methods: Fifty-six C4 knockout mice were ovariectomised (OVX) to eliminate the effect of endogenous feminine hormones followed by 17-β oestradiol (E2) administration in both dose- and time-dependent manners. Read More

    Selective Immunoglobulin M Deficiency Among Clozapine-Treated Patients: A Nested Case-Control Study.
    Prim Care Companion CNS Disord 2015 2;17(4). Epub 2015 Jul 2.
    Departments of Pharmacy (Dr Lozano) and Psychiatry (Drs Marin and Pascual and Ms Santacruz), Hospital Real de Nuestra Señora de Gracia, Zaragoza, Spain.
    Objective: To analyze the presence of selective immunoglobulin M immunodeficiency (SIgMD) among long-term clozapine-treated outpatients in a nested case-control study.

    Method: We investigated 33 patients who took clozapine and found 6 patients with SIgMD. These patients were compared with 67 patients not taking clozapine, of whom 2 had SIgMD. Read More

    Hypomorphic function and somatic reversion of DOCK8 cause combined immunodeficiency without hyper-IgE.
    Clin Immunol 2016 Feb 8;163:17-21. Epub 2015 Dec 8.
    Nuffield Department of Medicine, Experimental Medicine Division, University of Oxford, UK; Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford, UK.
    Loss-of-function mutations in DOCK8 are linked to hyper-IgE syndrome. Patients typically present with recurrent sinopulmonary infections, severe cutaneous viral infections, food allergies and elevated serum IgE. Although patients may present with a spectrum of disease-related symptoms, molecular mechanisms explaining phenotypic variability in patients are poorly defined. Read More

    CD4+CD25 high Foxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis.
    Hum Immunol 2016 Feb 7;77(2):196-200. Epub 2015 Dec 7.
    Translational Research Laboratory Prof. C. A. Hart, Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Recife, Brazil. Electronic address:
    Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Read More

    FSAP-mediated nucleosome release from late apoptotic cells is inhibited by autoantibodies present in SLE.
    Eur J Immunol 2016 Mar 22;46(3):762-71. Epub 2015 Dec 22.
    Department of Immunopathology, Sanquin Research, Amsterdam, The Netherlands, and Landsteiner Laboratory, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
    Inefficient clearance of apoptotic cells and the subsequent exposure of the immune system to nuclear contents are crucially involved in the pathogenesis of systemic lupus erythematosus (SLE). Factor VII-activating protease (FSAP) is activated in serum upon contact with dead cells, and releases nucleosomes from late apoptotic cells into the extracellular environment. We investigated whether FSAP-mediated nucleosome release from late apoptotic cells is affected in SLE patients. Read More

    IgG Suppresses Antibody Responses in Mice Lacking C1q, C3, Complement Receptors 1 and 2, or IgG Fc-Receptors.
    PLoS One 2015 30;10(11):e0143841. Epub 2015 Nov 30.
    Department of Medical Biochemistry and Microbiology, Uppsala University, Uppsala, Sweden.
    Antigen-specific IgG antibodies, passively administered to mice or humans together with large particulate antigens like erythrocytes, can completely suppress the antibody response against the antigen. This is used clinically in Rhesus prophylaxis, where administration of IgG anti-RhD prevents RhD-negative women from becoming immunized against RhD-positive fetal erythrocytes aquired transplacentally. The mechanisms by which IgG suppresses antibody responses are poorly understood. Read More

    Atherosclerosis Susceptibility in Mice Is Independent of the V1 Immunoglobulin Heavy Chain Gene.
    Arterioscler Thromb Vasc Biol 2016 Jan 12;36(1):25-36. Epub 2015 Nov 12.
    From the Department of Medicine and Center for Molecular Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden (M.C., D.N., K.A.P., D.K.J., G.K.H., D.F.J.K., S.M.); CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria (S.G., C.J.B.); and Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria (S.G., C.J.B.).
    Objective: The V1 (VHS107.1.42) immunoglobulin heavy chain gene is thought to be critical in producing IgM natural antibodies of the T15-idiotype that protect against both atherosclerosis and infection from Streptococcus pneumoniae. Read More

    Expression of activation-induced cytidine deaminase enhances the clearance of pneumococcal pneumonia: evidence of a subpopulation of protective anti-pneumococcal B1a cells.
    Immunology 2016 Jan;147(1):97-113
    Section of Allergy and Clinical Immunology, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.
    We describe a protective early acquired immune response to pneumococcal pneumonia that is mediated by a subset of B1a cells. Mice deficient in B1 cells (xid), or activation-induced cytidine deaminase (AID(-/-) ), or invariant natural killer T (iNKT) cells (Jα18(-/-) ), or interleukin-13 (IL-13(-/-) ) had impaired early clearance of pneumococci in the lung, compared with wild-type mice. In contrast, AID(-/-) mice adoptively transferred with AID(+/+) B1a cells, significantly cleared bacteria from the lungs as early as 3 days post infection. Read More

    Frequent IgG subclass and mannose binding lectin deficiency in patients with chronic fatigue syndrome.
    Hum Immunol 2015 Oct 30;76(10):729-35. Epub 2015 Sep 30.
    Institute of Medical Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany; Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Charité Universitätsmedizin Berlin, Berlin, Germany. Electronic address:
    Chronic fatigue syndrome (CFS) is a severe disease characterized by various symptoms of immune dysfunction. CFS onset is typically with an infection and many patients suffer from frequently recurrent viral or bacterial infections. Immunoglobulin and mannose binding lectin (MBL) deficiency are frequent causes for increased susceptibility to infections. Read More

    The prevalence of Selective Immunoglobulin M Deficiency (SIgMD) in Iranian volunteer blood donors.
    Hum Immunol 2016 Jan 30;77(1):7-11. Epub 2015 Sep 30.
    Immunology, Asthma, and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran; Department of Clinical Immunology and Allergy, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:
    Background: Selective Immunoglobulin M Deficiency (SIgMD) is known as a rare primary immunodeficiency characterized by an isolated deficiency of serum IgM. Other immunoglobulin levels and T-cell immunity are usually normal; although IgE may be elevated. SIgMD can be asymptomatic or with various bacterial and viral infections. Read More

    Antigen-specific IgA titres after 23-valent pneumococcal vaccine indicate transient antibody deficiency disease in children.
    Vaccine 2015 Nov 28;33(46):6320-6. Epub 2015 Sep 28.
    Department of Pediatric Immunology and Infectious Diseases/Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Lundlaan 6, 3508 AB Utrecht, The Netherlands. Electronic address:
    Paediatric patients with antibody deficiency may either be delayed in development of humoral immunity or may be persistently deficient in antibody production. To differentiate between these entities, we examined the 23-valent pneumococcal polysaccharide (PnPS) vaccine-induced IgM-, IgG- and IgA antibody responses in a cohort of 66 children with recurrent respiratory tract infections. Individual serum titres against 11 pneumococcal serotypes were measured by Luminex. Read More

    Mode of Bioenergetic Metabolism during B Cell Differentiation in the Intestine Determines the Distinct Requirement for Vitamin B1.
    Cell Rep 2015 Oct 24;13(1):122-31. Epub 2015 Sep 24.
    Division of Mucosal Immunology, Department of Microbiology and Immunology, The Institute of Medical Science, The University of Tokyo, Tokyo 108-8639, Japan; International Research and Development Center for Mucosal Vaccines, The Institute of Medical Science, The University of Tokyo, Tokyo 108-8639, Japan; Japan Science and Technology Agency (JST), Core Research for Evolutional Science and Technology (CREST), Tokyo 102-0076, Japan; Department of Medical Genome Science, Graduate School of Frontier Science, The University of Tokyo, Chiba 277-8562, Japan; Graduate School of Medicine, The University of Tokyo, Tokyo 113-0033, Japan.
    Bioenergetic metabolism varies during cell differentiation, but details of B cell metabolism remain unclear. Here, we show the metabolic changes during B cell differentiation in the intestine, where B cells differentiate into IgA(+) plasma cells (PCs). Naive B cells in the Peyer's patches (PPs) and IgA(+) PCs in the intestinal lamina propria (iLP) both used the tricarboxylic acid (TCA) cycle, but only IgA(+) PCs underwent glycolysis. Read More

    Acute Liver Injury Is Independent of B Cells or Immunoglobulin M.
    PLoS One 2015 25;10(9):e0138688. Epub 2015 Sep 25.
    MRC Centre for Inflammation Research, The University of Edinburgh, Edinburgh, United Kingdom; Clinical Surgery, The University of Edinburgh, Edinburgh, United Kingdom.
    Background & Aims: Acute liver injury is a clinically important pathology and results in the release of Danger Associated Molecular Patterns, which initiate an immune response. Withdrawal of the injurious agent and curtailing any pathogenic secondary immune response may allow spontaneous resolution of injury. The role B cells and Immunoglobulin M (IgM) play in acute liver injury is largely unknown and it was proposed that B cells and/or IgM would play a significant role in its pathogenesis. Read More

    Caspase-1 is required for maintenance of marginal zone B cells in pristane-induced lupus.
    Lupus 2016 Jan 24;25(1):81-7. Epub 2015 Sep 24.
    Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA
    Objective: Caspase-1 is required for nephritis and robust autoantibody development in the pristane model of murine lupus. The objective of this study was to evaluate the immune response and to study the splenic B and T cell populations in wild-type (WT) and caspase-1-/- mice following pristane injection in order to develop an understanding of why absence of caspase-1 is protective in pristane-induced lupus.

    Methods: Immunization responses to NP-Ficoll and NP-ovalbumin were assessed in WT and caspase-1-/- mice. Read More

    ATM deficiency promotes development of murine B-cell lymphomas that resemble diffuse large B-cell lymphoma in humans.
    Blood 2015 Nov 23;126(20):2291-301. Epub 2015 Sep 23.
    Experimental Immunology Branch, National Institute on Aging, National Institutes of Health, Bethesda, MD.
    The serine-threonine kinase ataxia-telangiectasia mutated (ATM) plays a central role in maintaining genomic integrity. In mice, ATM deficiency is exclusively associated with T-cell lymphoma development, whereas B-cell tumors predominate in human ataxia-telangiectasia patients. We demonstrate in this study that when T cells are removed as targets for lymphomagenesis and as mediators of immune surveillance, ATM-deficient mice exclusively develop early-onset immunoglobulin M(+) B-cell lymphomas that do not transplant to immunocompetent mice and that histologically and genetically resemble the activated B cell-like (ABC) subset of human diffuse large B-cell lymphoma (DLBCL). Read More

    Analysis of IgM antibody production and repertoire in a mouse model of Sjögren's syndrome.
    J Leukoc Biol 2016 Feb 17;99(2):321-31. Epub 2015 Sep 17.
    *Center for Oncology and Cell Biology, The Feinstein Institute for Medical Research, Manhasset, New York, USA; Division of Oral and Maxillofacial Pathology, Department of Dental Medicine, Long Island Jewish Medical Center, New Hyde Park, New York, USA; Department of Oral Biology, School of Dental Medicine, and Department of Biostatistics, State University of New York at Buffalo, Buffalo, New York, USA; Department of Dental Medicine and Medicine, Hofstra North Shore-LIJ School of Medicine, Hempstead, New York, USA; and Microarray Core Facility, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
    This study tested the hypothesis that B cells from salivary tissue are distinct in terms of proliferative capacity, immunoglobulin M secretion, repertoire, and autoantibody enrichment in Sjögren's syndrome. We sorted purified B cells from the spleen, cervical lymph nodes, and submandibular glands of a primary Sjögren's syndrome mouse model (Id3(-/-)). Enzyme-linked immunospot and proliferation assays were performed with stimulated B cells. Read More

    Altered B-cell subsets and functional B-cell defects in selective IgM deficiency.
    Clin Immunol 2015 Dec 3;161(2):96-102. Epub 2015 Sep 3.
    Institute for Medical Immunology, Charité University Medicine Berlin, Campus Virchow-Klinikum, Berlin, Germany; Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany.
    Primary selective IgM deficiency (sIgM) is characterized by diminished serum IgM, infections and autoimmunity. Although there is some evidence of B-cell defects the pathogenesis of sIgM is poorly understood. We determined peripheral B-cell subsets and IgM-expression levels in 31 adult sIgM patients by flow cytometry. Read More

    Generation of B cell-deficient pigs by highly efficient CRISPR/Cas9-mediated gene targeting.
    J Genet Genomics 2015 Aug 27;42(8):437-44. Epub 2015 May 27.
    State Key Laboratory of Reproductive Medicine and Jiangsu Key Laboratory of Xenotransplantation, Nanjing Medical University, Nanjing 210029, China. Electronic address:
    Generating B cell-deficient mutant is the first step to produce human antibody repertoires in large animal models. In this study, we applied the clustered regularly interspaced short palindromic repeat (CRISPR)/CRISPR-associated (Cas) system to target the JH region of the pig IgM heavy chain gene which is crucial for B cell development and differentiation. Transfection of IgM-targeting Cas9 plasmid in primary porcine fetal fibroblasts (PFFs) enabled inducing gene knock out (KO) in up to 53. Read More

    2B4 Is Dispensable for T-Dependent B Cell Immune Responses, but Its Deficiency Leads to Enhanced T-Independent Responses Due to an Increase in Peritoneal Cavity B1b Cells.
    PLoS One 2015 31;10(8):e0137314. Epub 2015 Aug 31.
    Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, Wisconsin, United States of America; Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America.
    The signaling lymphocyte activation molecule (SLAM) family plays important roles in adaptive immune responses. Herein, we evaluated whether the SLAM family member 2B4 (CD244) plays a role in immune cell development, homeostasis and antibody responses. We found that the splenic cellularity in Cd244-/- mice was significantly reduced due to a reduction in both CD4 T cells and follicular (Fo) B cells; whereas, the number of peritoneal cavity B cells was increased. Read More

    Validity of Primary Immunodeficiency Disease Diagnoses in United States Medicaid Data.
    J Clin Immunol 2015 Aug 14;35(6):566-72. Epub 2015 Aug 14.
    Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics and Epidemiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
    Purpose: Primary immunodeficiency diseases (PID) are a rare group of disorders with a wide array of clinical presentations. The absence of validated methods to identify these diseases in electronic databases has limited understanding of their epidemiology and the impact of drug therapies on outcomes. We measured the positive predictive values (PPVs) of ICD-9 diagnoses for identifying PID within US Medicaid. Read More

    Streptococcus pneumoniae antibody titres in patients with primary antibody deficiency receiving intravenous immunoglobulin (IVIG) compared to subcutaneous immunoglobulin (SCIG).
    Clin Exp Immunol 2015 Oct 29;182(1):51-6. Epub 2015 Jul 29.
    Department of Pediatrics, Allergy/Immunology Division, Jeffrey Modell Diagnostic Center for Primary Immunodeficiencies, Louisiana State University Health Sciences Center, New Orleans, LA, USA.
    Intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) are effective in the treatment of patients with primary antibody deficiency disorders (PAD). The purpose of this study was to evaluate Streptococcus pneumoniae (Spn) antibody titres to 14 serotypes in patients receiving IVIG compared to SCIG and to correlate Spn antibody levels to clinical outcome. The doses of immunoglobulin (Ig)G/kg/month were similar in both IVIG and SCIG groups. Read More

    Vitamin D Antibodies in Systemic Sclerosis Patients: Findings and Clinical Correlations.
    Isr Med Assoc J 2015 Feb;17(2):80-4
    Background: Vitamin D is a pivotal factor in calcium homeostasis and exerts immunomodulatory effects. Hypovitamin D has been demonstrated in systemic sclerosis (SSc) patients and may be related to more severe disease of longer duration and with extensive skin involvement.

    Objectives: To seek anti-vitamin D antibodies in SSc patients, as found by previous research in patients with systemic lupus erythematosus (SLE). Read More

    Genetic Interaction between Lyn, Ets1, and Btk in the Control of Antibody Levels.
    J Immunol 2015 Sep 24;195(5):1955-63. Epub 2015 Jul 24.
    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390; Department of Immunology, University of Texas Southwestern Medical Center, Dallas, TX 75390
    Tight control of B cell differentiation into plasma cells (PCs) is critical for proper immune responses and the prevention of autoimmunity. The Ets1 transcription factor acts in B cells to prevent PC differentiation. Ets1(-/-) mice accumulate PCs and produce autoantibodies. Read More

    Severe Autoimmune Hemolytic Anemia in an Infant Caused by Warm-reactive IGM and IGA Autoantibodies: A Case Report and Review of the Literature.
    J Pediatr Hematol Oncol 2015 Aug;37(6):468-71
    *Department of Hematology, St Jude Children's Research Hospital †Transfusion Services, Le Bonheur Children's Medical Center, Memphis, TN.
    Warm-reactive IgM autoimmune hemolytic anemia is uncommon and carries a poor prognosis in adults. There have been rare reports in children, generally associated with an underlying immunologic deficiency, and outcomes are quite variable. Warm IgM in combination with other antibodies has not been reported in children. Read More

    Processing of CD74 by the Intramembrane Protease SPPL2a Is Critical for B Cell Receptor Signaling in Transitional B Cells.
    J Immunol 2015 Aug 8;195(4):1548-63. Epub 2015 Jul 8.
    Biochemical Institute, Christian Albrechts University of Kiel, D-24118 Kiel, Germany;
    The invariant chain (CD74), a chaperone in MHC class II-mediated Ag presentation, is sequentially processed by different endosomal proteases. We reported recently that clearance of the final membrane-bound N-terminal fragment (NTF) of CD74 is mediated by the intramembrane protease signal peptide peptidase-like (SPPL)2a, a process critical for B cell development. In mice, SPPL2a deficiency provokes the accumulation of this NTF in endocytic vesicles, which leads to a B cell maturation arrest at the transitional 1 stage. Read More

    Deletion of WASp and N-WASp in B cells cripples the germinal center response and results in production of IgM autoantibodies.
    J Autoimmun 2015 Aug 2;62:81-92. Epub 2015 Jul 2.
    Department of Microbiology Tumor and Cell Biology, Karolinska Institutet, Stockholm 171 77, Sweden. Electronic address:
    Humoral immunodeficiency caused by mutations in the Wiskott-Aldrich syndrome protein (WASp) is associated with failure to respond to common pathogens and high frequency of autoimmunity. Here we addressed the question how deficiency in WASp and the homologous protein N-WASp skews the immune response towards autoreactivity. Mice devoid of WASp or both WASp and N-WASp in B cells formed germinal center to increased load of apoptotic cells as a source of autoantigens. Read More

    Comparison of FcRγ-Deficient and CD57+ Natural Killer Cells Between Cord Blood and Adult Blood in the Cytomegalovirus-Endemic Korean Population.
    Ann Lab Med 2015 Jul 21;35(4):423-8. Epub 2015 May 21.
    Department of Laboratory Medicine, Chonnam National University Medical School, Gwangju, Korea. ; Center for Creative Biomedical Scientists, Chonnam National University, Gwangju, Korea. ; Research Center for Cancer Immunotherapy, Chonnam National University Hwasun Hospital, Hwasun, Korea.
    Background: FcRγ-deficient natural killer (NK) cells (g(-)NK cells) have been associated with cytomegalovirus (CMV) infection. However, the frequency of g(-)NK cells in a CMV-endemic area (i.e. Read More

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