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Research Article Free access | 10.1172/JCI113349

B lymphocytes from X-linked agammaglobulinemia. Delayed expression of light chain and demonstration of Lyonization in carriers.

J Schwaber, J Payne, and R Chen

Immunology Division, Children's Hospital, Boston, MA 02115.

Find articles by Schwaber, J. in: JCI | PubMed | Google Scholar

Immunology Division, Children's Hospital, Boston, MA 02115.

Find articles by Payne, J. in: JCI | PubMed | Google Scholar

Immunology Division, Children's Hospital, Boston, MA 02115.

Find articles by Chen, R. in: JCI | PubMed | Google Scholar

Published February 1, 1988 - More info

Published in Volume 81, Issue 2 on February 1, 1988
J Clin Invest. 1988;81(2):514–522. https://doi.org/10.1172/JCI113349.
© 1988 The American Society for Clinical Investigation
Published February 1, 1988 - Version history
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Abstract

We report an unusual phenotype of B cells in a patient with X-linked agammaglobulinemia (XLA), and cellular evidence for Lyonization of B cells from his mother and sister. The patient has a failure of B cell maturation at the stage of early B lymphocytes, associated with production of D(mu delta) H chain. The phenotype of his B cells includes: (a) limitation to expression of the mu and delta H chain isotypes, (b) production of mu and delta H chains of reduced size and (c) delayed expression of L chain. Peripheral blood and B cell lines from the patient's mother and sister include 50% cells that express H chain without L chain. B cell lines from the mother and sister produce full-length mu and gamma H chains and truncated mu and delta chains corresponding to the H chains produced by the patient's B cells. Clones with normal and XLA phenotype have been isolated from B cell lines derived from the patient's mother. We conclude that the dimorphism of mother's and sister's B cells results from Lyonization, implying that the gene defect in XLA is intrinsic to B lymphocytes.

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