Selective deficiency of CD4+/CD45RA+ lymphocytes in patients with ataxia-telangiectasia

Roberto Paganelli, Enrico Scala, Elisa Scarselli, Claudio Ortolani, Andrea Cossarizza, Daniela Carmini, Fernando Aiuti, Massimo Fiorilli

Research output: Contribution to journalArticle

39 Citations (Scopus)

Abstract

Several immunological abnormalities have been observed in ataxia-telangiectasia (AT), the most consistent being defects of immunoglobulin isotypes, decreased T-cell numbers, and reduced proliferative responses to mitogens. We examined the distribution of T lymphocytes expressing distinctive surface Ag characteristic of "naive" (CD45RA+) and "memory" (CD29+, CD45RO+) T cells, in both CD4+ and CD8+ (bright and dim) lymphocytes from 13 AT patients, compared with healthy agematched controls. We found that, irrespective of age, patients with AT had a severe deficiency of CD4+/CD45RA+ lymphocytes. This decrease accounted for the reduction of total CD4+ cells, since the absolute numbers of memory CD4+ cells were not significantly different in AT and in controls. Functional tests revealed poor proliferative responses to phytohemagglutinin and normal responses to soluble Ag (tetanus toxoid) in AT patients. These data fit with the distribution of naive and memory cells, which are known to respond predominantly to mitogens or to recall Ag, respectively. CD45RA molecules were normally expressed on CD8+ lymphocytes. This rules out a generalized defect of regulation or differential splicing as the cause of defective expression of CD45RA on CD4+ cells. The selective deficiency of CD4+CD45RA+ may provide a cellular basis for some functional T-cell abnormalities of AT patients. Furthermore, it might practically serve for an early, or even prenatal, diagnosis of this disease.

Original languageEnglish
Pages (from-to)84-91
Number of pages8
JournalJournal of Clinical Immunology
Volume12
Issue number2
DOIs
Publication statusPublished - Mar 1992

Fingerprint

Ataxia Telangiectasia
Lymphocytes
T-Lymphocytes
Mitogens
Tetanus Toxoid
Immunoglobulin Isotypes
Phytohemagglutinins
Prenatal Diagnosis
Cell Count

Keywords

  • Ataxia-telangiectasia
  • CD45RA+ cells
  • mitogen and antigen T-cell responses

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Paganelli, R., Scala, E., Scarselli, E., Ortolani, C., Cossarizza, A., Carmini, D., ... Fiorilli, M. (1992). Selective deficiency of CD4+/CD45RA+ lymphocytes in patients with ataxia-telangiectasia. Journal of Clinical Immunology, 12(2), 84-91. https://doi.org/10.1007/BF00918137

Selective deficiency of CD4+/CD45RA+ lymphocytes in patients with ataxia-telangiectasia. / Paganelli, Roberto; Scala, Enrico; Scarselli, Elisa; Ortolani, Claudio; Cossarizza, Andrea; Carmini, Daniela; Aiuti, Fernando; Fiorilli, Massimo.

In: Journal of Clinical Immunology, Vol. 12, No. 2, 03.1992, p. 84-91.

Research output: Contribution to journalArticle

Paganelli, R, Scala, E, Scarselli, E, Ortolani, C, Cossarizza, A, Carmini, D, Aiuti, F & Fiorilli, M 1992, 'Selective deficiency of CD4+/CD45RA+ lymphocytes in patients with ataxia-telangiectasia', Journal of Clinical Immunology, vol. 12, no. 2, pp. 84-91. https://doi.org/10.1007/BF00918137
Paganelli, Roberto ; Scala, Enrico ; Scarselli, Elisa ; Ortolani, Claudio ; Cossarizza, Andrea ; Carmini, Daniela ; Aiuti, Fernando ; Fiorilli, Massimo. / Selective deficiency of CD4+/CD45RA+ lymphocytes in patients with ataxia-telangiectasia. In: Journal of Clinical Immunology. 1992 ; Vol. 12, No. 2. pp. 84-91.
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