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POLR3A DNA-dependent RNA polymerase catalyzes the transcription of DNA into RNA using the four ribonucleoside triphosphates as substrates. Largest and catalytic core component of RNA polymerase III which synthesizes small RNAs, such as 5S rRNA and tRNAs. Forms the polymerase active center together with the second largest subunit. A single stranded DNA template strand of the promoter is positioned within the central active site cleft of Pol III. A bridging helix emanates from RPC1 and crosses the cleft near the catalytic site and is thought to promote translocation of Pol III by acting as a ratchet that moves the RNA-DNA hybrid through the active site by switching from straight to bent conformations at each step of nucleotide addition. Plays a key role in sensing and limiting infection by intracellular bacteria and DNA viruses. Acts as nuclear and cytosolic DNA sensor 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. Defects in POLR3A are the cause of leukodystrophy hypomyelinating type 7 with or without oligodontia and/or hypogonadotropic hypogonadism (HLD7). An autosomal recessive neurodegenerative disorder characterized by childhood onset of progressive motor decline manifest as spasticity, ataxia, tremor, and cerebellar signs, as well as mild cognitive regression. Other features may include hypodontia or oligodontia and hypogonadotropic hypogonadism. There is considerable inter- and intrafamilial variability. Belongs to the RNA polymerase beta' chain family. Note: This description may include information from UniProtKB.
Protein type: EC; Nucleotide Metabolism - purine; Nucleotide Metabolism - pyrimidine; Transcription initiation complex; Transferase
Chromosomal Location of Human Ortholog: 10q22.3
Cellular Component: cytosol; DNA-directed RNA polymerase III complex; membrane; nucleoplasm
Molecular Function: chromatin binding; DNA binding; DNA-directed RNA polymerase activity; metal ion binding
Biological Process: defense response to virus; innate immune response; positive regulation of interferon type I production; positive regulation of interferon-beta production; transcription from RNA polymerase III promoter; transcription, DNA-dependent
Disease: Leukodystrophy, Hypomyelinating, 7, With Or Without Oligodontia And/or Hypogonadotropic Hypogonadism
Reference #:  O14802 (UniProtKB)
Alt. Names/Synonyms: DNA-directed RNA polymerase III largest subunit; DNA-directed RNA polymerase III subunit A; DNA-directed RNA polymerase III subunit RPC1; hRPC155; POLR3A; polymerase (RNA) III (DNA directed) polypeptide A, 155kDa; RNA polymerase III 155 kDa subunit; RNA polymerase III subunit C1; RNA polymerase III subunit C160; RNA polymerase III subunit RPC155-D; RPC1; RPC155
Gene Symbols: POLR3A
Molecular weight: 155,642 Da
Basal Isoelectric point: 8.76  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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