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Journal of Clinical Immunology

, Volume 11, Issue 5, pp 262–267 | Cite as

Expression of immunoglobulin genes in common variable immunodeficiency

  • Hideo Kaneko
  • Naomi Kondo
  • Fumiaki Motoyoshi
  • Seiji Mori
  • Yuki Kobayashi
  • Yuko Inoue
  • Tadao Orii
Original Articles

Abstract

Five common variable immunodeficiency (CVI) patients were analyzed for expression of immunoglobulin (Ig) genes. In the pokeweed mitogen (PWM)-induced Ig-production assay, the combination of T and B cells showed that all patients' T cells had normal helper functions and all patients' B cells had profound defects. The defective B-cell maturation stages based on their Ig gene expression patterns were variable. One of five patients showed normal μ-chain gene expression and nearly normal IgM production, but neither IgG nor IgA production, which suggested that this patient's B-cell defects might lie on a μ- to γ or μ- to α class-switch stage. B cells in another patient showed low μ-chain gene expression and low IgM production, but an Ig enhancer region, which is an important region for expression of Ig genes, was intact. Thus, this patient might have a transacting factor defect which interacts with the Ig enhancer region. The other three patients showed no μ-chain gene expression and no IgM production. Thus, their B-cell defects lay on the B-cell maturation stage, similar to X-linked agammaglobulinemia. These results showed that primary B-cell defects in CVI occurred at several B-cell differentiation stages, which could be recognized by expression of Ig genes.

Key words

Common variable immunodeficiency (CVI) μ messenger RNA interleukins Ig enhancer 

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Copyright information

© Plenum Publishing Corporation 1991

Authors and Affiliations

  • Hideo Kaneko
    • 1
  • Naomi Kondo
    • 1
  • Fumiaki Motoyoshi
    • 1
  • Seiji Mori
    • 1
  • Yuki Kobayashi
    • 1
  • Yuko Inoue
    • 1
  • Tadao Orii
    • 1
  1. 1.Department of PediatricsGifu University School of MedicineGifuJapan

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