Conditions d'induction de la pathologie immune du syndrome de Griscelli dans la souris ashen déficiente en Rab27a et étude du rôle de Rab37 dans les lymphocytes cytotoxiques

Griscelli syndrome is characterized by hypopigmentation associated with primitive neurological impairment resulting from myosin 5a defect in type 1 (GS1), and in type 2 (GS2) with a deregulation of the immune response resulting from defect in Rab27a, a protein required for the cytotoxic activity of lymphocytes. Our work has tried to define the experimental conditions allowing the induction, in the ashen murine model, of the immune disturbance known as hemophagocytic syndrome, which characterized GS2. Infection with Listeria Monocytogenes of the ashen mutant mice, which spontaneously occurred on the genetic background C3H, did not allow reproducing the features of the hemophagocytic syndrome. The Rab27a mutation was then transferred to the C57B1/6 and Balb/c genetic backgrounds. The experimental conditions using LCMV infection of the ashen mice on C57B1/6 background were recently settled, which allowed inducing the first signs of the hemophagocytic syndrome as observed in patients.