CC2D1A (coiled-coil and C2 domain-containing 1A), also known as Freud-1, Aki1 or TAPE (TBK1-associated protein in endolysosomes), is a evolutionary conserved protein located in different subcellular compartments, including the nucleus, centrosome, and endolysosomes. It acts as a scaffold protein that interacts with various proteins and plays diverse biological roles. Mutations in CC2D1A have been linked to nonsyndromic mental retardation(NMSR) and generate a truncated 85-kDa product. Several isoforms of CC2D1A proteins have also been described: 120 /130-kDa doublet of long isoform and 67 kDa short isoform. The short isoform of CC2D1A has been identified as the predominant isoform in rodent cells, while the long isoform is more abundant in human cells. Recently it has been reported that CC2D1A/TAPE is the first innate immune regulator implicated in both TLR and RLR signaling at a very early step.