Ontology highlight
ABSTRACT:
SUBMITTER: Hsieh WC
PROVIDER: S-EPMC5792625 | biostudies-literature | 2018 Jan
REPOSITORIES: biostudies-literature
Hsieh Wan-Chen WC Hsu Tzu-Sheng TS Chang Ya-Jen YJ Lai Ming-Zong MZ
Nature communications 20180131 1
X-linked lymphoproliferative syndrome type-2 (XLP-2) is a primary immunodeficiency disease attributed to XIAP mutation and is triggered by infection. Here, we show that mouse Xiap<sup>-/-</sup> regulatory T (Treg) cells and human XIAP-deficient Treg cells are defective in suppressive function. The Xiap<sup>-/-</sup> Treg cell defect is linked partly to decreased SOCS1 expression. XIAP binds SOCS1 and promotes SOCS1 stabilization. Foxp3 stability is reduced in Xiap<sup>-/-</sup> Treg cells. In ad ...[more]