DGCR6 is a developmental regulatory gene located in the DiGeorge critical region of chromosome 22, deleted in 22q11.2 deletion syndrome (22q11DS) 1. The gene exists in two functional copies (DGCR6 and DGCR6L) on chromosome 22, with the duplication dating back at least 12 million years, suggesting evolutionary selective pressure for maintaining both paralogs 2. DGCR6 encodes a ~25 kDa nuclear phosphoprotein with predominantly nuclear localization 3. Functionally, DGCR6 shows high expression in embryonic brain, spinal cord, and pharyngeal arches 1, with neural crest cell-restricted expression in mature tissues 4. The gene acts as a repressor of TBX-1 and UFD1L expression in developing neural crest derivatives, and DGCR6 attenuation in neural crest cells produces cardiovascular anomalies characteristic of DiGeorge syndrome 4. Clinically, DGCR6 expression dysregulation correlates with anxiety disorders in 22q11DS children, independent of genomic imprinting but driven by other epigenetic mechanisms 5. Additionally, DGCR6 deletion associates with autism spectrum disorders and schizophrenia, implicating this gene in broader neurodevelopmental pathways 6.