| First Author | Kinjyo I | Year | 2010 |
| Journal | J Immunol | Volume | 185 |
| Issue | 12 | Pages | 7151-5 |
| PubMed ID | 21076068 | Mgi Jnum | J:167466 |
| Mgi Id | MGI:4868320 | Doi | 10.4049/jimmunol.1003193 |
| Citation | Kinjyo I, et al. (2010) Cutting edge: Lymphoproliferation caused by Fas deficiency is dependent on the transcription factor eomesodermin. J Immunol 185(12):7151-5 |
| abstractText | A hallmark of autoimmune lymphoproliferative syndrome (ALPS), caused by mutation of the Fas death receptor, is massive lymphadenopathy from aberrant expansion of CD4(-)CD8(-) (double-negative [DN]) T cells. Eomesodermin (Eomes) is a member of the T-box family of transcription factors and plays critical roles in effector cell function and memory cell fitness of CD8(+) T lymphocytes. We provide evidence in this study that DN T cells exhibit dysregulated expression of Eomes in humans and mice with ALPS. We also find that T cell-specific deletion of Eomes prevents lymphoid hypertrophy and accumulation of DN T cells in Fas-mutant mice. Although Eomes has critical physiological roles in the function and homeostasis of CD8(+) T cells, overexpression of Eomes appears to enable pathological induction or expansion of unusual CD8-related T cell subsets. Thus, antagonism of Eomes emerges as a therapeutic target for DN T cell ablation in ALPS. |
