Microprocessor complex subunit DGCR8

Microprocessor complex subunit DGCR8 is a protein that in humans is encoded by the DGCR8 gene, which is located on chromosome 22. This protein plays a crucial role in the microprocessor complex, essential for microRNA (miRNA) biogenesis. The process of miRNA biogenesis is critical for the regulation of gene expression at the post-transcriptional level. DGCR8, in conjunction with Drosha, another core component of the microprocessor complex, is involved in the initial step of miRNA processing in the nucleus.

Function

The primary function of DGCR8 is to assist in the production of miRNAs, small non-coding RNA molecules that play a key role in regulating gene expression. DGCR8 does this by binding to primary miRNA transcripts (pri-miRNAs) and facilitating their cleavage by Drosha, an RNAse III enzyme. This cleavage results in the formation of precursor miRNA (pre-miRNA), which is then exported from the nucleus and further processed into mature miRNA. These mature miRNAs are capable of regulating gene expression by binding to complementary sequences on target messenger RNAs (mRNAs), leading to their degradation or inhibition of translation.

Structure

DGCR8 contains two double-stranded RNA (dsRNA) binding domains, which are essential for its interaction with pri-miRNAs. The structure of DGCR8 is critical for its function, as it determines the specificity and efficiency of pri-miRNA processing. Mutations or alterations in the DGCR8 gene can disrupt the normal structure and function of the protein, potentially leading to various diseases.

Clinical Significance

Alterations in the DGCR8 gene have been associated with several disorders, including DiGeorge syndrome, a genetic disorder that results in poor development of several body systems. Research has also suggested a link between DGCR8 and certain types of cancer, as miRNAs processed by the microprocessor complex can act as oncogenes or tumor suppressors. Understanding the role of DGCR8 in miRNA biogenesis has been crucial for developing potential therapeutic strategies targeting miRNA regulation in various diseases.

Genetic Information

The DGCR8 gene is located on the long (q) arm of chromosome 22 at position 11.2, within a region commonly associated with DiGeorge syndrome. This location is significant because deletions or mutations in this region can lead to the symptoms associated with the syndrome, highlighting the importance of DGCR8 in normal physiological development and function.

See Also

• MicroRNA
• Drosha
• DiGeorge Syndrome
• Non-coding RNA
• Gene expression