Journal article

Effects of a Small-Molecule Perforin Inhibitor in a Mouse Model of CD8 T Cell-Mediated Neuroinflammation

C Gonzalez-Fierro, C Fonte, E Dufourd, V Cazaentre, S Aydin, B Engelhardt, RR Caspi, B Xu, G Martin-Blondel, JA Spicer, JA Trapani, J Bauer, RS Liblau, C Bost

Neurology Neuroimmunology and Neuroinflammation | LIPPINCOTT WILLIAMS & WILKINS | Published : 2023

Open access

Abstract

Background and ObjectivesAlteration of the blood-brain barrier (BBB) at the interface between blood and CNS parenchyma is prominent in most neuroinflammatory diseases. In several neurologic diseases, including cerebral malaria and Susac syndrome, a CD8 T cell-mediated targeting of endothelial cells of the BBB (BBB-ECs) has been implicated in pathogenesis.MethodsIn this study, we used an experimental mouse model to evaluate the ability of a small-molecule perforin inhibitor to prevent neuroinflammation resulting from cytotoxic CD8 T cell-mediated damage of BBB-ECs.ResultsUsing an in vitro coculture system, we first identified perforin as an essential molecule for killing of BBB-ECs by CD8 T c..

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University of Melbourne Researchers

Grants

Awarded by Institut National de la Santé et de la Recherche Médicale


Funding Acknowledgements

This work was supported by grants from Agence Nationale pour la Recherche CE17-0014 (RSL), Fondation pour la Recherche Medicale (Prix Line Pomaret Delalande [CGF]; DEQ20170336727 [RSL]) ARSEP-French MS society: ARSEP 2019-20 (RSL), BETPSY RHU 18- RHUS-0012(RSL), Austrian Science Fund FWF: P34864-B (JB). The early stages of perforin inhibitor drug development were funded by the Strategic Drug Discovery Initiative (SDDI) Program of the Wellcome Trust UK (JAT).