Ganglioside-Induced Defective Granule Exocytosis-Mediated Cytotoxicity in Hemophagocytic Lymphohistiocytosis
Sasa Radoja PhD
Children’s National Medical Center – Washington, District of Columbia USA
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Hemophagocytic lymphohistiocytosis (HLH) is a life threatening immune disorder that usually presents during infancy or early childhood. Dysfunction of a particular type of immune cells, called “killer cells” is thought to be the basis for development of HLH. The levels of a factor, a type of lipid called ganglioside, are known to be elevated in blood of HLH patents. Previous studies have suggested that the increased levels of this lipid factor cause dysfunction of the killer cells, which in turn contributes to the development of HLH disease. The goal of our studies proposed here is to determine exactly how this lipid factor depresses the function of the killer cells. We believe that findings originating from the studies will allow us to better understand how this disease develops and eventually help us find more efficient ways for its treatment.