POLR3F
Domain
The [4FE-4S] cluster-binding domain adopts a globular structure that serves as an interaction hub that connects the POLR3C/RPC3-POLR3F/RPC6-POLR3G/RPC7 heterotrimer to the Pol III core.
Function
DNA-dependent RNA polymerase catalyzes the transcription of DNA into RNA using the four ribonucleoside triphosphates as substrates (PubMed:20413673, PubMed:21358628, PubMed:33558764, PubMed:34675218). Specific peripheric component of RNA polymerase III (Pol III) which synthesizes small non-coding RNAs including 5S rRNA, snRNAs, tRNAs and miRNAs from at least 500 distinct genomic loci. Part of POLR3C/RPC3-POLR3F/RPC6-POLR3G/RPC7 heterotrimer that coordinates the dynamics of Pol III stalk and clamp modules during the transition from apo to elongation state (PubMed:20413673, PubMed:33558764, PubMed:33558766). Pol III plays a key role in sensing and limiting infection by intracellular bacteria and DNA viruses, including varicella zoster virus. Acts as a nuclear and cytosolic DNA sensor detecting AT-rich DNA, involved in innate immune response. Can sense non-self dsDNA that serves as template for transcription into dsRNA. The non-self RNA polymerase III transcripts, such as Epstein-Barr virus-encoded RNAs (EBERs) induce type I interferon and NF-kappa-B through the RIG-I pathway (PubMed:19609254, PubMed:19631370, PubMed:30211253). Preferentially binds double-stranded DNA (dsDNA) (PubMed:21358628).
Involvement in disease
Immunodeficiency 101, varicella zoster virus-specific
IMD101
An autosomal dominant immunologic disorder characterized by reactivation of varicella zoster virus (VZV) infection in adulthood after primary childhood infection with VZV. The viral reactivation manifests as central nervous system vasculitis with stroke-like episodes and lacunar infarcts on brain imaging. Features include headache, hemiparesis, impaired balance, and other neurologic signs.
None
The disease may be caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the eukaryotic RPC34/RPC39 RNA polymerase subunit family.
Cellular localization
- Nucleus
Alternative names
DNA-directed RNA polymerase III subunit RPC6, RNA polymerase III subunit C6, DNA-directed RNA polymerase III subunit F, RNA polymerase III 39 kDa subunit, RPC39, POLR3F