DGCR8 Mouse mAb

DGCR8 (DiGeorge Syndrome Critical Region 8), also known as Pasha in invertebrates, is an essential double-stranded RNA-binding protein that functions as a core component of the Microprocessor complex alongside the RNase III enzyme Drosha, playing a pivotal role in the canonical biogenesis of microRNAs (miRNAs) within the nucleus. By recognizing and binding to the apical loop and single-stranded RNA segments of primary miRNA transcripts (pri-miRNAs), DGCR8 serves as a molecular ruler that positions Drosha to precisely cleave the pri-miRNA base, thereby generating precursor miRNAs (pre-miRNAs) that are subsequently exported to the cytoplasm for further processing into mature miRNAs involved in post-transcriptional gene regulation. Beyond its central function in miRNA processing, DGCR8 contributes to the maintenance of genomic stability, DNA damage response, and cellular differentiation, and its haploinsufficiency due to deletions in chromosome 22q11.2 is directly linked to DiGeorge syndrome (22q11.2 deletion syndrome), a multisystem developmental disorder characterized by congenital heart defects, immune deficiencies, and neuropsychiatric abnormalities, underscoring the critical importance of precise DGCR8-mediated regulatory networks in human development and disease.