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Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion.
J Schwaber, … , N Koenig, J Girard
J Schwaber, … , N Koenig, J Girard
Published October 1, 1988
Citation Information: J Clin Invest. 1988;82(4):1471-1476. https://doi.org/10.1172/JCI113754.
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Research Article

Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion.

Abstract

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.

Authors

J Schwaber, N Koenig, J Girard

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