Search

Your search keyword '"Simpson, Pippa"' showing total 14 results
Start Over Author "Simpson, Pippa" Journal journal of pediatric gastroenterology & nutrition
14 results on '"Simpson, Pippa"'

14. Intact Regulatory T-Cell Function but Defective Generation of IL–17A-Producing CD4+T Cells in XIAP Deficiency

Author

Gurram, Bhaskar, Hammelev, Erin, Syverson, Grant, Haribhai, Dipica, Yan, Key, Simpson, Pippa, Salzman, Nita, and Verbsky, James W.

Abstract

X-linked inhibitor of apoptosis (xIAP) deficiency is a primary immune deficiency disorder associated with hemophagocytic lymphohistiocytosis. About 17% of xIAP-deficient patients present with very early onset severe colitis with high mortality. We hypothesized that xIAP deficiency leads to defective generation and/or survival of T regulatory cells (Treg) through its involvement in transforming growth factor-ß signaling. We used a T-cell transfer model of chronic colitis and observed a mild increase in colitis severity induced by naïve CD4+T cells from xIAP0/-mice compared with colitis induced by naïve CD4+T cells from WT mice. We did not observe any significant difference in the induction of Tregcells in these studies. We next tested whether xIAP is required for Tregcell function by co-transferring xIAP-/0or WT Tregcells with naïve WT CD4 cells in this model. We demonstrate that XIAP-deficient Tregcells were able to prevent disease similarly to WT Tregcells. In these experiments we, however, found a significantly decreased percentage of IL–17A-producing CD4+T cells in mice receiving Tregsfrom xIAP0/-mice. xIAP appears dispensable for the generation of induced Tregcells as well as function of natural Tregcells. There appeared to be a role of xIAP in generation of IL–17-producing cells from either naïve CD4+T cells or Tregcells. Further research is needed to explore the role of xIAP in generation of IL–17-producing cells.

Published
2016
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results