In animals, pri-miRNAs are processed by the RNase III enzyme Drosha [14] in the nucleus which is assisted by the DiGeorge syndrome chromosomal region 8 (DGCR8) in humans [15–17] and Pasha in D. melanogaster and C. elegans [17,18]. The gene discussed is DROSHA; the disease is 22q11.2 deletion syndrome.