Immunity
Volume 2, Issue 3, March 1995, Pages 223-238
Journal home page for Immunity

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

https://doi.org/10.1016/1074-7613(95)90047-0Get rights and content
Under an Elsevier user license
open archive

Abstract

The common γ chain (γc) of the IL-2, IL-4, IL-7, IL-9, and IL-15 receptors is defective in humans with XSCID. Mice lacking γc expression had poplastic thymuses; the thymocytes responded to γc-independent mitogens, but not γc-dependent 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, γδ intestinal intraepithelial lymphocytes, dendritic epidermal T cells, peripheral lymph nodes, and gut-associated lymphoid tissue were absent. These findings underscore the Importance of γc in lymphoid development. Moreover, differences in humans and mice lacking γc expression indicate species-specific differences in the roles of γc-dependent cytokines or in the existence of redundant pathways. These mice provide an important model for studying the pathophysiology of and gene therapy for human XSCID.

Cited by (0)

9

Current address: Anatomy Department, Monash University, Wellington Road, Clayton, 3168, Victoria, Australia