Oral Presentation The 4th Prato Conference on Pore Forming Proteins 2018

Novel small molecule inhibitors of lymphocyte perforin (#9)

Joseph Trapani 1 2 , Hedieh Akhlaghi 1 2 , Vivien Sutton 1 2 , Ilia Voskoboinik 1 2 , Kate Gartlan 3 , Geoff R Hill 3 , Meike Welz 4 , Wolfgang Kastenmuller 4 , David Middlemiss 5 , Julie Spicer 6
  1. Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, VIC, Australia
  2. Cancer Immunology Program, Peter MacCallum Cancer Centre, Melabourne, VIC, Australia
  3. QIMR Berghofer Medical Research Institute , Brisbane, QLD, Australia
  4. Institute of Experimental Immunology , University of Bonn, Bonn, Germany
  5. XaviaPharm, Hertfordshire , UK
  6. Auckland Cancer Society Research Centre, Faculty of Medical and Health Sciences, University of Auckland , Auckland, NZ

Perforin is a pore-forming protein that regulates the entry of pro-apoptotic granzymes into cells targeted by cytotoxic T lymphocytes and natural killer cells. As the product of a single copy gene that enables granzyme access to their intra-cellular substrate, perforin is potentially an excellent target for the development of focused immunosuppressive drugs. Our group has developed a number of lead chemical series that potently inhibit perforin-mediated target cell death, as exemplified in a variety of in vitro assays and pre-clinical mouse models. Data will be presented showing that small molecule peforin inhibitors protect mouse bone marrow stem cells against allogeneic destruction and the survival of hepatocytes targeted by auto-reactive cytotoxic T lymphocytes in mice infected with hepatotropic adenovirus. In the latter model, short term (2 day) perforin inhibition delivered I/P was highly effective at protecting mice against fulminant liver failure resulting from adenovirus-induced hepatitis.