In the nucleus, these pri-miRNAs are processed by RNase III Drosha and the DiGeorge syndrome chromosomal region 8, Microprocessor Complex Subunit (DGCR8; DiGeorge syndrome chromosomal region 8) protein, generating a precursor miRNA (pre-miRNA) of approximately 100 bp. The gene discussed is DROSHA; the disease is 22q11.2 deletion syndrome.