Article
Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3).
Cell - 14 Nov 2003
Feldmann Jérôme, Callebaut Isabelle, Raposo Graça, Certain Stéphanie, Bacq Delphine, Dumont Cécile, Lambert Nathalie, Ouachée-Chardin Marie, Chedeville Gaëlle, Tamary Hannah, Minard-Colin Véronique, Vilmer Etienne, Blanche Stéphane, Le Deist Françoise, Fischer Alain, de Saint Basile Geneviève
Abstract excerpt
Secretion of cytolytic granules content at the immunological synapse is a highly regulated process essential for lymphocyte cytotoxicity. This process requires the rapid transfer of perforin containing lytic granules to the target cell interface, followed by their docking and fusion with the plasma membrane. Defective cytotoxicity characterizes a genetically heterogeneous condition named familial hemophagocytic...
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