In 2016 we made, through our partnership with Sparks, the following award:
Professor Christian Münz and Dr. Lynn Wong, University of Zürich, Switzerland. £50,000 over two years.
Mutations in X chromosome-linked inhibitor of apoptosis protein (XIAP) have been found in patients with X-linked lymphoproliferative disease (XLP2). These reduce the levels of XIAP and presumably compromise Epstein Barr virus (EBV) specific immune control during primary infection, leading to often fatal lymphoproliferative disease during primary EBV infection of XLP2 patients.
The proposed research project is designed to understand how XIAP inhibition compromises EBV specific immune control and tumorigenesis in the only small animal in vivo model of this infection.
This knowledge should indicate which immune compartments have to be therapeutically strengthened or even adoptively transferred into XLP2 patients in order for them to survive primary EBV infection.