In humans, the loss of one functional allele of DGCR8 is sufficient to impair miRNA production, suggesting to be the cause of the physiological defects seen in 22q11.2 deletion syndrome, including pluripotency defects, immunodeficiency due to an underdeveloped thymus, and psychiatric illnesses (Colomer-Boronat et al., 2025; McDonald-McGinn et al., 2015). Here, DGCR8 is linked to immune system disorder.