title: Defective anti-polysaccharide antibody response in patients with ataxia-telangiectasia.
abstract: The immunodeficiency in ataxia-telangiectasia (A-T) patients involves both cellular and humoral immunity; however, the specific antibody response is not well defined. Frequent respiratory infections are a prominent feature in A-T. Streptococcus pneumoniae is a common pathogen responsible for these infections. Defective B cell membrane signaling has been reported in A-T cells. These observations prompted us to investigate the B cell response to six frequently encountered pneumococcal serotypes in A-T patients. We found defective IgG antibody production to all studied serotypes (3, 6B, 7F, 14, 19F, and 23F) in 22 of 31 A-T patients (71%) who were immunized with a polyvalent pneumococcal vaccine. The impaired antibody responses did not correlate with either history of infection or serum immunoglobulin isotype levels. In addition, we did not observe any correlation between the pneumococcal antibody production and a specific mutation or level of intracellular ATM (ataxia-telangiectasia mutated) protein in lysates of lymphoblastoid cell lines from these patients. Our results suggest that the extent and severity of the recurrent sinopulmonary infections may depend not only on the immunological defects but also on other ATM-dependent physiological responses.
authors.affiliation: Department of Molecular Biology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Ozden
authors.initials: O
authors.lastname: Sanal
authors.forename: Filiz
authors.initials: F
authors.lastname: Ozbaş-Gerçeker
authors.forename: Leman
authors.initials: L
authors.lastname: Yel
authors.forename: Fügen
authors.initials: F
authors.lastname: Ersoy
authors.forename: Ilhan
authors.initials: I
authors.lastname: Tezcan
authors.forename: A Izzet
authors.initials: AI
authors.lastname: Berkel
authors.forename: Ayşe
authors.initials: A
authors.lastname: Metin
authors.forename: Richard A
authors.initials: RA
authors.lastname: Gatti
mesh_terms: D000293:Adolescent; D000328:Adult; D000907:Antibodies, Bacterial / Q000276:immunology*; D001260:Ataxia Telangiectasia / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004268:DNA-Binding Proteins / Q000235:genetics; D018450:Disease Progression; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D011008:Pneumococcal Infections / Q000276:immunology; D022242:Pneumococcal Vaccines; D013296:Streptococcus pneumoniae; D014157:Transcription Factors
chemical_list: D000907:Antibodies, Bacterial; D004268:DNA-Binding Proteins; D007074:Immunoglobulin G; D022242:Pneumococcal Vaccines; C087296:TRIM29 protein, human; D014157:Transcription Factors