Final Activity Report Summary - CYTOLYTIC SYNAPSE (Regulation of cytotoxic T lymphocyte activity at the lytic synapse)
Each researcher within the Team (1 early-stage researcher and 3 experienced researchers) was in charge of a specific project. The main scientific achievements obtained are the following: WASP regulates the molecular organisation and the stability of the IS; WASP regulates CTL cytotoxic activity, in particular against tumoural B cells ; tumoural B cells activate an abnormal actin remodelling program to regulate their motility. In addition, we provide a novel analytical method to study lytic granule composition and contribute to the preclinical validation of gene therapy for WAS.
This project illustrates how the study of a rare disease as a model can yield data that are relevant way beyond the specific disease studied. Indeed, the study of a WAS patient with a secondary mutation was instructive in that it represents a case of 'natural gene therapy'. Our expertise on molecular regulation of actin cytoskeleton remodelling in lymphocytes from WAS patients was used to investigate how malignant lymphocytes remodel their actin cytoskeleton as a 'strategy' to migrate and invade tissues.