RNase HII-B Activators

Ribonuclease H2, subunit B (RNase H2B), is an essential component of the RNase H2 enzyme complex, which plays a pivotal role in DNA replication and repair. Specifically, RNase H2B contributes to the excision of ribonucleotide monophosphates (rNMPs) embedded in DNA, an activity crucial for maintaining genomic stability. The RNase H2 complex is unique in its ability to recognize and cleave these rNMPs from DNA/RNA hybrids and is involved in the DNA replication process by removing the RNA primers and initiating Okazaki fragment maturation.

Mutations in the gene coding for RNase H2B can lead to a rare autosomal recessive disorder known as Aicardi-Goutières syndrome (AGS), characterized by early-onset encephalopathy and chronic cerebrospinal inflammation, highlighting the enzyme's significance in immune response regulation and neurological development.RNase H2B's activity is not confined to the nucleus; it also localizes to the mitochondria, indicating a role in mitochondrial DNA replication and repair. Its function is integral to the blocking of genome instability, which is a hallmark of aging and many diseases. The enzyme operates in a coordinated manner with other repair and replication machinery, ensuring the integrity of the genetic material is preserved during cell division.