https://www.selleckchem.com/products/pki587.html
Familial hemophagocytic lymphohistiocytosis type III (FHL3) is a rare disease caused by mutations to the UNC13D gene and the subsequent absence or decreased activity of the Munc13-4 protein. Munc13-4 is essential for the exocytosis of perforin and granzyme containing granules from cytotoxic cells. Without it, these cells are able to recognize an immunological insult but are unable to execute their cytotoxic functions. The result is a hyper-inflammatory state that, if left untreated, is fatal. Currently, the only curative treatment is hem