DiGeorge Syndrome Critical Region Gene 8 (DGCR8)

DGCR8 interacted directly with Drosha, and the 2 proteins were present within a complex of about 650 kD. Both DGCR8 and Drosha could homodimerize in the absence of single-stranded RNA (ssRNA) in addition to interacting with each other. Depletion of DGCR8 from HeLa cells with small interfering RNA led to accumulation of pri-miRNA and loss of pre-miRNA and mature miRNA.

The deduced 773-amino acid protein contains a WW motif in its N-terminal half and 2 double-stranded RNA-binding motifs in its C-terminal half. In situ hybridization of mouse embryos revealed Dgcr8 expression in neuroepithelium of primary brain, limb bud, vessels, thymus, and palate during various developmental stages.