Protocol

Immune Homeostasis

Volume 979 of the series Methods in Molecular Biology pp 189-197

Date:

Flow Cytometric Measurement of SLAM-Associated Protein and X-Linked Inhibitor of Apoptosis

  • Rebecca A. MarshAffiliated withDivision of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Center Email author 
  • , Jack J. BleesingAffiliated withDivision of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Center
  • , Alexandra H. FilipovichAffiliated withDivision of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Center

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Abstract

Flow cytometry is a valuable tool for the detection and characterization of proteins expressed by individual cells. Flow cytometry can be used to measure cell expression of 2 intracellular proteins that are involved in the regulation of immune homeostasis, SLAM-associated protein (SAP) and X-linked inhibitor of apoptosis (XIAP). These proteins are defective in patients with the immune deficiency X-linked lymphoproliferative disease (XLP), due to mutations in the SH2D1A and XIAP/BIRC4 genes, respectively (Coffey et al. Nat Genet 20:129-135 1998; Nichols et al. Proc Natl Acad Sci U S A 95:13765-13770, 1998; Sayos et al. Nature 395:462-469, 1998; Rigaud et al. Nature 444:110-114, 2006). This procedure describes a technique that can be efficiently used to detect SAP and XIAP by flow cytometry.

Key words

X-linked Lymphoproliferative Disease (XLP) SLAM-Associated Protein (SAP) X-linked Inhibitor of Apoptosis (XIAP)