Folding into a characteristic stem-loop structure [7], the pri-miRNA binds to the double-stranded RNA-binding domain (dsRBD) of the protein known as the DiGeorge syndrome critical region of gene 8 (DGCR8) and is then cleaved by the ribonuclease III Drosha (also known as RNASEN), which releases an intermediate stem-loop structure of 60–70 nt known as the pre-miRNA. This evidence concerns the gene DROSHA and 22q11.2 deletion syndrome.