Defective lymphoid development in mice lacking expression of the common cytokine receptor γ chain

X Cao, EW Shores, J Hu-Li, MR Anver, BL Kelsail… - Immunity, 1995 - cell.com
X Cao, EW Shores, J Hu-Li, MR Anver, BL Kelsail, SM Russell, J Drago, M Noguchi…
Immunity, 1995cell.com
The common y chain (yc) of the IL-2, 11-4, IL-7, IL-g, and IL-15 receptors is defective in
humans with XSCID. Mice lacking ys expression had hypoplastic thymuses; the thymocytes
responded to ye-independent mitogens, but not y&ependent stimuli. Splenic T cells were
diminished at 3 weeks of age, but CD4+ T cells markedly increased by 4 weeks. B cells were
greatly diminished, in contrast with the situation in XSCID. NK cells, y6 intestinal
intraepithelial lymphocytes, dendritic epidermal T cells, peripheral lymph nodes, and gut …
Summary
The common y chain (yc) of the IL-2, 11-4, IL-7, IL-g, and IL-15 receptors is defective in humans with XSCID. Mice lacking ys expression had hypoplastic thymuses; the thymocytes responded to ye-independent mitogens, but not y&ependent stimuli. Splenic T cells were diminished at 3 weeks of age, but CD4+ T cells markedly increased by 4 weeks. B cells were greatly diminished, in contrast with the situation in XSCID. NK cells, y6 intestinal intraepithelial lymphocytes, dendritic epidermal T cells, peripheral lymph nodes, and gut-associated lymphoid tissue were absent. These findings underscore the importance of T= In lymphoid development. Moreover, differences in humans and mice lacking Te expression indicate species-specific differences in the roles of yc-dependent cytokines or in the existence of redundant pathways. These mice provide an impotiant model for studying the pathophysiology of and gene therapy for human XSCID.
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