DGCR8 Structure and Biological Function

DGCR8 (DiGeorge syndrome critical region 8), a non-coding RNA-binding protein, comprises the Rhed (RNA-binding heme domain), along with two dsRBD (double-stranded RNA binding domains) and the CTT (C-terminal tail). DGCR8 participates in miRNA synthesis by forming a microprocessor with DROSHA and directing DROSHA to cleave at the correct position of pri-miRNA. The advancement of research has unveiled numerous non-canonical functions of DGCR8. Due to its extensive involvement in crucial processes such as noncoding RNA synthesis, alternative splicing of mRNA, and post-transcriptional regulation, deficiency of DGCR8 leads to a diverse array of developmental abnormalities. Additionally, apart from its close association with DiGeorge syndrome, DGCR8 exhibits dysregulation in various malignancies and disorders, playing a crucial role in the migration, invasion, metastasis of cancer cells and the pathogenesis of diverse diseases. This review provides a comprehensive overview of the structural characteristics and diverse biological functions of DGCR8, with particular emphasis on its pivotal roles in regulating cell proliferation, differentiation, apoptosis, senescence, body growth and development, as well as a variety of diseases including cancer. These findings unveil the potential of DGCR8 may be as a therapeutic target for congenital developmental defects and associated tumors and other diseases, offering novel insights for the treatment of related disorders.

CSTR: 32200.14.cjcb.2025.01.0015