Abstract

MONGE-RODRIGUEZ, Silvia Leticia  and  ESPINOZA-MORA, María del Rosario. Exocytosis mechanism of cytotoxic granules: keys given from primary hemophagocytic lymphohistiocytosis. Acta bioquím. clín. latinoam. [online]. 2017, vol.51, n.1, pp.123-131. ISSN 0325-2957.

Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of hyperinflammation, in which the immune response is highly stimulated but it is ineffective. In primary HLH, the exocytosis process of cytotoxic granules or the effector proteins contained there are altered and the activation process of cytotoxic cells could be affected as well. During exocytosis there is dysfunction in vesicle maturation or translocation, in regulator proteins of the docking and priming process, or in v-SNARE and t-SNARE complexes. Connection between the cytotoxic cell and the target cell may be compromised if perforin effector protein is affected. SAP and XIAP have a role in the activation of immune cells. Though there is currently much known about the molecules participating in cytotoxicity, there is redundancy in protein functions involved in primary HLH, and there are some functions of these proteins that are still unknown.

Keywords : Linfohistiocitosis hemofagocítica; Exocitosis; Gránulos citotóxicos; Hemophagocytic lymphohistiocytosis; Exocitosis; Cytotoxic granules.

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