The mechanism of antibody production using the model of primary immunodeficiency, especially IgA deficiency

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IgA deficiency (IgAD) is the most common immunodeficiency, but the pathogenesis of most cases of IgAD is poorly understood. The gene and protein expression levels of members of the IgA subclasses in IgAD patients were analyzed by a reverse transcriptase (RT) -PCR method that could differentiate between α 1 and α 2 gene expression. Three selective, 5 partial and 2 secondary IgAD patients were examined. Peripheral blood mononuclear cells that were unstimulated or stimulated with TGF- β 1 and PMA for 24 hours were cultured. The IgAl/IgA2 expression ratios were measured by zone densitometry. Three bands appeared (the α 1 and α2 genes, and a hetero-duplex formation), owing to the difference of 39 bases between α 1 and α2 mRNAs. In controls, there were no significant differences in the IgAl/IgA2 ratios between unstimulated and stimulated cells. In selective IgAD patients, both α1 and α2 gene expression were induced following stimulation, and α1 gene expression was induced more dominantly than in the other IgAD patients following stimulation. Based on our results, it was thought that suppression of α 1 gene expression may be related to the pathogenesis of IgAD.