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Aberrant Patterns of Immunoglobulin Levels in Wiskott‐Aldrich Syndrome
Author(s) -
INOUE R.,
KONDO N.,
KUWABARA N.,
ORII T.
Publication year - 1995
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.1995.tb03552.x
Subject(s) - peripheral blood mononuclear cell , isotype , microbiology and biotechnology , biology , exon , antibody , immunoglobulin class switching , immunoglobulin heavy chain , dna methylation , wiskott–aldrich syndrome , immunoglobulin m , methylation , b cell , immunology , epigenetics , gene , immunoglobulin g , gene expression , monoclonal antibody , genetics , in vitro
We have investigated IgM deficiency in Wiskott‐Aldrich syndrome patients. From the assessment of T and B cell functions in pokeweed mitogen‐induced immunoglobulin ( Ig ) production. IgM deficiency was chiefly thought to result from B cell dysfunction. The percentages of surface IgM ‐bearing cells were decreased in peripheral blood mononuclear cells ( PBMCs ) and the number of IgM ‐secreting cells was also decreased. Lymphoblast cell lines ( LCLs ) from the patients have produced IgG and IgA , but never IgM . Moreover the expression of the Cμ transcript from the patients' PBMCs and their LCLs were decreased, whereas the Cμ gene was well expressed. No germ‐line polymorphism existed between the patients and the controls in the Cμ region, and no mutation was detected in the μs C‐terminal and the M exon by nucleotide sequencing. These suggest that the Ig heavy chain ( IGHC ) isotype switch may be abnormally accelerated in the patients' B cells. While the methylation patterns of the human Ig enhancer gene region were the same between the patient and the control, the methylation patterns of the Iγ1 region showed less methylation in the patient than in the control, which may cause low IgM expression and high expression levels of other classes located downstream of the IGHC gene.