Selective deficiency of immunoglobulin A2.
E Van Loghem, BJ Zegers, EJ Bast… - The Journal of clinical …, 1983 - Am Soc Clin Investig
E Van Loghem, BJ Zegers, EJ Bast, L Kater
The Journal of clinical investigation, 1983•Am Soc Clin InvestigA case of familial selective IgA2 deficiency is described. The mother had no detectable IgA2,
but a low level of IgA1. She had anti-alpha 2 antibodies of the IgG class. One of her
daughters also lacked IgA2 with a normal level of IgA1. The analysis of the immunoglobulin
haplotypes of the family suggested the deletion of the alpha 2-gene. In addition, the analysis
of B lymphocytes of mother and daughter showed the absence of IgA2-bearing cells. Upon
stimulation with pokeweed mitogen, the B cells differentiated into IgA1-containing plasma …
but a low level of IgA1. She had anti-alpha 2 antibodies of the IgG class. One of her
daughters also lacked IgA2 with a normal level of IgA1. The analysis of the immunoglobulin
haplotypes of the family suggested the deletion of the alpha 2-gene. In addition, the analysis
of B lymphocytes of mother and daughter showed the absence of IgA2-bearing cells. Upon
stimulation with pokeweed mitogen, the B cells differentiated into IgA1-containing plasma …
A case of familial selective IgA2 deficiency is described. The mother had no detectable IgA2, but a low level of IgA1. She had anti-alpha 2 antibodies of the IgG class. One of her daughters also lacked IgA2 with a normal level of IgA1. The analysis of the immunoglobulin haplotypes of the family suggested the deletion of the alpha 2-gene. In addition, the analysis of B lymphocytes of mother and daughter showed the absence of IgA2-bearing cells. Upon stimulation with pokeweed mitogen, the B cells differentiated into IgA1-containing plasma cells, but IgA2-containing cells were not found. The results suggest a defect in the generation of intraclonal B cell isotype diversity. The molecular basis of this phenomenon is unknown.
The Journal of Clinical Investigation