Skip to main content
NCBI home page
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1987 May;68(2):392–396.

Serum IgG subclass deficiency in ataxia-telangiectasia.

P Aucouturier 1, C Bremard-Oury 1, C Griscelli 1, M Berthier 1, J L Preud'homme 1
PMCID: PMC1542718  PMID: 3652519

Abstract

Serum IgG subclass levels were determined using a competitive indirect immunoenzymatic assay with monoclonal antibodies in 16 patients with ataxia-telangiectasia. Eight children had IgA deficiency, two had IgG and IgA deficiency and six patients showed no immunoglobulin class abnormality. However, IgG4 and IgG2 levels were undetectable or low in almost every patient. An IgG3 deficiency was associated with the IgG2-IgG4 defect in three patients with undetectable IgA. IgG1 was very low in one patient with a total IgG deficiency. There was no clear correlation between subclass levels and the occurrence of infections.

Full text

PDF
392

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aucouturier P., Couderc L. J., Gouet D., Danon F., Gombert J., Matheron S., Saimot A. G., Clauvel J. P., Preud'homme J. L. Serum immunoglobulin G subclass dysbalances in the lymphadenopathy syndrome and acquired immune deficiency syndrome. Clin Exp Immunol. 1986 Jan;63(1):234–240. [PMC free article] [PubMed] [Google Scholar]
  2. Aucouturier P., Danon F., Daveau M., Guillou B., Sabbah A., Besson J., Preud'homme J. L. Measurement of serum IgG4 levels by a competitive immunoenzymatic assay with monoclonal antibodies. J Immunol Methods. 1984 Nov 16;74(1):151–162. doi: 10.1016/0022-1759(84)90376-4. [DOI] [PubMed] [Google Scholar]
  3. Bach J. F. The thymus in immunodeficiency diseases: new therapeutic approaches. Birth Defects Orig Artic Ser. 1983;19(3):245–253. [PubMed] [Google Scholar]
  4. Cunningham-Rundles C., Oxelius V. A., Good R. A. IgG2 and IgG3 subclass deficiencies in selective IgA deficiency in the United States. Birth Defects Orig Artic Ser. 1983;19(3):173–175. [PubMed] [Google Scholar]
  5. Epstein W. L., Fudenberg H. H., Reed W. B., Boder E., Sedgwick R. P. Immunologic studies in ataxia-telangiectasia. I. Delayed hypersensitivity and serum immune globulin levels in probands and first-degree relatives. Int Arch Allergy Appl Immunol. 1966;30(1):15–29. [PubMed] [Google Scholar]
  6. Lane P. J., MacLennan I. C. Impaired IgG2 anti-pneumococcal antibody responses in patients with recurrent infection and normal IgG2 levels but no IgA. Clin Exp Immunol. 1986 Aug;65(2):427–433. [PMC free article] [PubMed] [Google Scholar]
  7. Martinez-Alonso C., Coutinho A., Augustin A. A. Immunoglobulin C-gene expression. I. The commitment to IgG subclass of secretory cells is determined by the quality of the nonspecific stimuli. Eur J Immunol. 1980 Sep;10(9):698–702. doi: 10.1002/eji.1830100908. [DOI] [PubMed] [Google Scholar]
  8. Mongini P. K., Paul W. E., Metcalf E. S. T cell regulation of immunoglobulin class expression in the antibody response to trinitrophenyl-ficoll. Evidence for T cell enhancement of the immunoglobulin class switch. J Exp Med. 1982 Mar 1;155(3):884–902. doi: 10.1084/jem.155.3.884. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Out T. A., van Munster P. J., De Graeff P. A., Thé T. H., Vossen J. M., Zegers B. J. Immunological investigations in individuals with selective IgA deficiency. Clin Exp Immunol. 1986 Jun;64(3):510–517. [PMC free article] [PubMed] [Google Scholar]
  10. Oxelius V. A., Berkel A. I., Hanson L. A. IgG2 deficiency in ataxia-telangiectasia. N Engl J Med. 1982 Mar 4;306(9):515–517. doi: 10.1056/NEJM198203043060905. [DOI] [PubMed] [Google Scholar]
  11. Oxelius V. A. IgG subclass levels in infancy and childhood. Acta Paediatr Scand. 1979 Jan;68(1):23–27. doi: 10.1111/j.1651-2227.1979.tb04424.x. [DOI] [PubMed] [Google Scholar]
  12. Oxelius V. A., Laurell A. B., Lindquist B., Golebiowska H., Axelsson U., Björkander J., Hanson L. A. IgG subclasses in selective IgA deficiency: importance of IgG2-IgA deficiency. N Engl J Med. 1981 Jun 11;304(24):1476–1477. doi: 10.1056/NEJM198106113042408. [DOI] [PubMed] [Google Scholar]
  13. Rivat-Peran L., Buriot D., Salier J. P., Rivat C., Dumitresco S. M., Griscelli C. Immunoglobulins in ataxia-telangiectasia: evidence for IgG4 and IgA2 subclass deficiencies. Clin Immunol Immunopathol. 1981 Jul;20(1):99–110. doi: 10.1016/0090-1229(81)90166-5. [DOI] [PubMed] [Google Scholar]
  14. Waldmann T. A., Broder S., Goldman C. K., Frost K., Korsmeyer S. J., Medici M. A. Disorders of B cells and helper T cells in the pathogenesis of the immunoglobulin deficiency of patients with ataxia telangiectasia. J Clin Invest. 1983 Feb;71(2):282–295. doi: 10.1172/JCI110768. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Yount W. J. IgG2 deficiency and ataxia-telangiectasia. N Engl J Med. 1982 Mar 4;306(9):541–543. doi: 10.1056/NEJM198203043060911. [DOI] [PubMed] [Google Scholar]
  16. Zegers B. J., van der Giessen M., Reerink-Brongers E. E., Stoop J. W. The serum IgG subclass levels in healthy infants of 13--62 weeks of age. Clin Chim Acta. 1980 Feb 28;101(2-3):265–269. doi: 10.1016/0009-8981(80)90252-1. [DOI] [PubMed] [Google Scholar]
  17. van der Giessen M., Rossouw E., van Veen T. A., van Loghem E., Zegers B. J., Sander P. C. Quantification of IgG subclasses in sera of normal adults and healthy children between 4 and 12 years of age. Clin Exp Immunol. 1975 Sep;21(3):501–509. [PMC free article] [PubMed] [Google Scholar]

Articles from Clinical and Experimental Immunology are provided here courtesy of British Society for Immunology

RESOURCES