Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion.
The X chromosome-linked antibody deficiency disease, X-linked agammaglobulinemia ( XLA), results from failure of B lymphoid development. In the minor form of XLA, B lymphoid development terminates at the stage of immature B lymphocytes that produce truncated Ig heavy (H) chains composed of D-J-C(mu/delta), resulting from failure of VH gene rearrangement. Fusion of B cells from a patient with the minor form of XLA with mouse myeloma results in complementation of this defect; hybrid cells produce full-length H chains composed of VH-D-JH-C. The VH gene is of human origin. Complementation occurs independent of retention or loss of the human X ( XLA) chromosome in the hybrid cells. These results indicate that the D-JH-C structure of the XLA B cells is fully functional for the subsequent rearrangement of a VH gene element, and that failure of immunoglobulin expression is susceptible to correction.[1]References
- Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion. Schwaber, J., Koenig, N., Girard, J. J. Clin. Invest. (1988) [Pubmed]
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