Hyper-immunoglobulin M syndromes caused by intrinsic B-lymphocyte defects.

@article{Durandy2005HyperimmunoglobulinMS,
  title={Hyper-immunoglobulin M syndromes caused by intrinsic B-lymphocyte defects.},
  author={Anne H Durandy and Patrick Revy and Kohsuke Imai and Alain Fischer},
  journal={Immunological reviews},
  year={2005},
  volume={203},
  pages={
          67-79
        }
}
Hyper-immunoglobulin M (IgM) syndromes are primary immunodeficiencies characterized by normal or elevated serum IgM levels with the absence of other isotypes, pinpointing to a defect in the Ig class switch recombination (CSR). The delineation of hyper-IgM syndromes made it possible to better define the mechanisms underlying the two major events of antibody maturation in humans, CSR and introduction of somatic hypermutation (SHM) in the variable region of immunoglobulins. The description of the… CONTINUE READING

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