UNC13D (Protein unc-13 homolog D) variants and mutations

UNC13D (also known as Protein unc-13 homolog D) is a human protein-coding gene encoding a protein unc-13 homolog D protein. It primes cytotoxic granules for membrane fusion in natural-killer cells and cytotoxic T cells, enabling release of perforin and granzymes. Biallelic loss-of-function variants cause familial hemophagocytic lymphohistiocytosis type 3 with uncontrolled immune activation. This analysis covers 1,629 UNC13D variants and mutations. Of these, 77% have computational variant effect predictions. Disease context includes Familial hemophagocytic lymphohistiocytosis, autoinflammatory syndrome, and hereditary hemophagocytic lymphohistiocytosis. Example UNC13D variants include A2V, L5F, and L5P.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable UNC13D variants

Examples include A2V, L5F, L5P, S6F, H7Y, P8L, Q10H, R11C. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.