A dominant-negative IFNGR1 variant reveals broad immune cell sequestering of IFN-γ
Samantha Chan, Mai B. Margetts, Longfei Wang, Jack Godsell, Josh Chatelier, Belinda Liu, Charlotte A. Slade, Andrew Brett, Kasha P. Singh, Vanessa L. Bryant, Lauren J. Howson
Samantha Chan, Mai B. Margetts, Longfei Wang, Jack Godsell, Josh Chatelier, Belinda Liu, Charlotte A. Slade, Andrew Brett, Kasha P. Singh, Vanessa L. Bryant, Lauren J. Howson
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Research Letter Genetics Immunology

A dominant-negative IFNGR1 variant reveals broad immune cell sequestering of IFN-γ

Abstract

Authors

Samantha Chan, Mai B. Margetts, Longfei Wang, Jack Godsell, Josh Chatelier, Belinda Liu, Charlotte A. Slade, Andrew Brett, Kasha P. Singh, Vanessa L. Bryant, Lauren J. Howson

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Figure 1

IFN-γR1 is ubiquitously overexpressed and sequesters IFN-γ on the surface of patients’ cells.

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IFN-γR1 is ubiquitously overexpressed and sequesters IFN-γ on the surfac...
(A) Familial segregation of the IFNGR1 c.817delA (p.I273fs) variant. (B) Expression of IFN-γR1 on PBMC subsets. (C) pSTAT1 staining of PBMCs stimulated with IFN-γ and gated on monocytes. (D) PBMCs were cultured with LPS and a 10-fold dilution series of IFN-γ. (E) RNA-seq of differentially expressed immune genes. Surface IFN-γ detected on (F) monocytes and (G) PBMC subsets following incubation with IFN-γ. (H) IFN-γ dissociation from monocytes over time. The line represents nonlinear regression and the dashed line the dissociation half-life. Error bars represent SD between technical duplicates. HD, healthy donor; MAIT, mucosal-associated invariant T (cell). NS, no stimulation; P, patient.