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Expression of immunoglobulin genes in common variable immunodeficiency.

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 mu-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 mu- to gamma or mu- to alpha class-switch stage. B cells in another patient showed low mu-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 mu-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.

journal_name

J Clin Immunol

journal_title

Journal of clinical immunology

authors

Kaneko H,Kondo N,Motoyoshi F,Mori S,Kobayashi Y,Inoue Y,Orii T

doi

10.1007/BF00918184

subject

Has Abstract

pub_date

1991-09-01 00:00:00

pages

262-7

issue

5

eissn

0271-9142

issn

1573-2592

journal_volume

11

pub_type

杂志文章

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