Ribonuclease H2 subunit B (RNASEH2B)

Ribonuclease H2 subunit B is an essential non-catalytic subunit of the RNase H2 enzyme complex, which, together with subunits A and C, is responsible for removing misincorporated ribonucleotides from DNA and degrading RNA/DNA hybrids formed during replication and transcription. Its key structural feature is a C-terminal PIP box motif, allowing interaction with proliferating cell nuclear antigen (PCNA) and localization to DNA replication foci, thus facilitating genome surveillance and repair. Mutations in RNASEH2B disrupt ribonucleotide excision repair and cause Aicardi-Goutières syndrome, a severe neuroinflammatory and autoimmune disease characterized by immune system activation due to accumulated DNA damage and faulty nucleic acid metabolism. This subunit does not possess enzymatic activity by itself but is critical for the function and stability of the RNase H2 complex.

Other names

Ribonuclease H2, subunit BRNase H2 subunit BRibonuclease HI subunit BDLEU8AGS2FLJ11712Deleted in lymphocytic leukemia 8Aicardi-Goutières syndrome 2 proteinRNH2B_HUMAN

For a hypothetical inhibitor: prevention of ribonucleotide excision repair, increased genome instability, or modulation of immune response For genetic rescue/modification: restoration of ribonucleotide excision function, reduction of R-loop accumulation