Transcription of intergenic miRs leads to the formation of primary miRs (pri-miR) with a characteristic hairpin or stem-loop structure (Denli et al., 2004), which are subsequently processed by the nuclear RNase III, Drosha (Zeng et al., 2005), and its partner proteins, among which there is the DiGeorge Syndrome Critical Region 8 (DGCR8, known as Pasha in invertebrates), named for its association with DiGeorge Syndrome (Shiohama et al., 2003; Roth et al., 2013), to become precursor miR s (pre-miR). Here, DROSHA is linked to 22q11.2 deletion syndrome.