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UNC13D (unc-13 homolog D) encodes the protein Munc13-4, a member of the UNC13 family. This protein plays a critical role in immune cell function, particularly in cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. The primary function of Munc13-4 is to regulate the exocytosis of cytolytic granules—specialized vesicles containing proteins that induce target cell death—by facilitating their maturation, docking, priming, and fusion with the plasma membrane at immunological synapses. Mutations or deficiencies in UNC13D cause severe immune dysregulation, notably Familial Hemophagocytic Lymphohistiocytosis Type 3 (FHL3).
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