How do SAP family deficiencies compromise immunity?

Trends in Immunology
Zhongjun Dong, André Veillette

Abstract

The signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) family of adaptors couples SLAM family receptors to activating intracellular signaling pathways that drive immune cell activation or differentiation. In the absence of SAP family adaptors, SLAM family receptors become inhibitory, possibly through coupling to the Src-homology-2-containing phosphatases. This "switch-of-function" of SLAM family receptors provides an explanation for the severe immune dysfunctions observed in humans with X-linked lymphoproliferative disease due to SAP deficiency, as well as in genetically engineered mice that lack SAP family adaptors.

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Citations

Feb 15, 2011·Annual Review of Immunology·Shane Crotty
Oct 13, 2011·Cellular and Molecular Life Sciences : CMLS·Elena SieniMaurizio Aricò
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Jul 12, 2019·Frontiers in Immunology·Evelyn Gerth, Jochen Mattner
Mar 27, 2019·Cellular & Molecular Immunology·Shasha Chen, Zhongjun Dong
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Jan 25, 2020·Molecular Cancer Research : MCR·Mariko IshibashiHideto Tamura
May 1, 2021·Antibody Therapeutics·Mi DengCheng Cheng Zhang

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