DNA-directed RNA polymerase III subunit H (POLR3H)

DNA-directed RNA polymerase III subunit H (POLR3H) is a protein-coding subunit of the multi-protein RNA polymerase III complex, essential for the transcription of a variety of small non-coding RNAs including tRNAs, 5S rRNA, U6 snRNA, and other short RNAs fundamental to RNA maturation and cellular translation machinery. It is a peripheral part of the polymerase III complex, forms a mobile stalk (with CRCP/RPC9), and is crucial for transcription initiation. POLR3H also serves as a nuclear and cytosolic DNA sensor and plays a role in the innate immune system by detecting cytosolic non-self double-stranded DNA, which can trigger type I interferon and NF-κB signaling via the RIG-I pathway, particularly in response to certain viral infections. Mutations in POLR3H and other Pol III subunits are linked to tissue-specific genetic disorders including hypomyelinating leukodystrophy, cerebellar-retinal degeneration, and potentially increase susceptibility to some infections[1][2][3][6]. No direct pharmacological modulators or drugs targeting POLR3H are currently identified.

Other names

DNA-directed RNA polymerase III subunit RPC8RNA polymerase III subunit C8KIAA1665RPC22.9C25RNA polymerase III subunit 22.9 kDa subunitRNA nucleotidyltransferase (DNA-directed)polymerase (RNA) III (DNA directed) polypeptide H (22.9kD)polymerase (RNA) III subunit H