B Cell Immunodeficiency

Science's STKE  26 Nov 2002:
Vol. 2002, Issue 160, pp. tw447-TW447
DOI: 10.1126/stke.2002.160.tw447

Several cytokine receptors utilize the common cytokine receptor γ chain (γc), and mutations in γc result in the condition known as X-linked severe combined immunodeficiency (XSCID). Lack of signaling through the interleukin-7 (IL-7) and IL-15 receptors leads to a loss of natural killer and T cells in XSCID patients, although the cause of the B cell defect is not established. Ozaki et al. observed that B cells from mice lacking IL-21 receptor had altered antibody production. When combined with a loss of IL-4 signaling, a severely impaired immunoglobulin G response was observed that is strikingly similar to that seen in XSCID individuals.

K. Ozaki, R. Spolski, C. G. Feng, C.-F. Qi, J. Cheng, A. Sher, H. C. Morse III, C. Liu, P. L. Schwartzberg, W. J. Leonard, A critical role for IL-21 in regulating immunoglobulin production. Science 298, 1630-1634 (2002). [Abstract] [Full Text]