de SAINT BASILE Geneviève : UMR_S1163 : Normal ans pathological homestasis onf the Immune system

Unit : INSERM UMR_S1163 - Institut Imagine - Université Paris Descartes

Institut Imagine
Laboratoire Homéostasie normale et pathologique du système immunitaire
24 Bd du Montparnasse
75015 PARIS

Speciality : Department IMMUNO

Director of the unit : Alain FISCHER

Principal investigator :

de SAINT BASILE Geneviève
Email : genevieve.de-saint-basileSPAMFILTER@inserm.fr Phone number: +33 (0)1 42 75 43 35

Composition of research team :

  • de SAINT BASILE Geneviève (DRE-INSERM, HDR)
  • MENASCHE Gaël (CR1-NSERM, HDR)

5 Recent publications of the research team :

Sepulveda, F.E., Debeurme, F., Menasche, G., Kurowska, M., Cote, M., Pachlopnik Schmid, J., Fischer, A., and de Saint Basile, G. (2013). Distinct severity of HLH in both human and murine mutants with complete loss of cytotoxic effector PRF1, RAB27A, and STX11. Blood 121:595-603.

Pachlopnik Schmid, J., Lemoine, R., Nehme, N., Cormier-Daire, V., Revy, P., Debeurme, F., Debre, M., Nitschke, P., Bole-Feysot, C., Legeai-Mallet, L., et al. (2012). Polymerase epsilon1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature ("FILS syndrome"). J Exp Med 209:2323-2330.

Kurowska, M., Goudin, N., Nehme, N.T., Court, M., Garin, J., Fischer, A., de Saint Basile, G., and Menasche, G. (2012). Terminal transport of lytic granules to the immune synapse is mediated by the kinesin-1/Slp3/Rab27a complex. Blood 119:3879-3889.

De Saint Basile, G., Menasche, G., and Fischer, A. (2010). Molecular mechanisms of biogenesis anD exocytosis of cytotoxic granules. Nat Rev Immunol 10:568-579.

Cote, M., Menager, M.M., Burgess, A., Mahlaoui, N., Picard, C., Schaffner, C., Al-Manjomi, F., Al-Harbi, M., Alangari, A., Le Deist, F., et al. (2009). Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells. J Clin Invest 119:3765-3773.

Menager, M.M., Menasche, G., Romao, M., Knapnougel, P., Ho, C.H., Garfa, M., Raposo, G., Feldmann, J., Fischer, A., and de Saint Basile, G. (2007). Secretory cytotoxic granule maturation and exocytosis require the effector protein hMunc13-4. Nat Immunol 8:257-267.