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POLR3B DNA-dependent RNA polymerase catalyzes the transcription of DNA into RNA using the four ribonucleoside triphosphates as substrates. Second largest core component of RNA polymerase III which synthesizes small RNAs, such as 5S rRNA and tRNAs. Proposed to contribute to the polymerase catalytic activity and forms the polymerase active center together with the largest subunit. Pol III is composed of mobile elements and RPC2 is part of the core element with the central large cleft and probably a clamp element that moves to open and close the cleft. 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 POLR3B are the cause of leukodystrophy, hypomyelinating, type 8, with or without oligodontia and/or hypogonadotropic hypogonadism (HLD8). An autosomal recessive neurodegenerative disorder characterized by early childhood onset of cerebellar ataxia and mild intellectual disabilities associated with diffuse hypomyelination apparent on brain MRI. Variable features include oligodontia and/or hypogonadotropic hypogonadism. 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: 12q23.3
Cellular Component: cytosol; DNA-directed RNA polymerase III complex; nucleoplasm
Biological Process: positive regulation of innate immune response; positive regulation of interferon type I production; positive regulation of interferon-beta production
Disease: Hypogonadotropic Hypogonadism 7 With Or Without Anosmia; Leukodystrophy, Hypomyelinating, 7, With Or Without Oligodontia And/or Hypogonadotropic Hypogonadism; Leukodystrophy, Hypomyelinating, 8, With Or Without Oligodontia And/or Hypogonadotropic Hypogonadism
Reference #:  Q9NW08 (UniProtKB)
Alt. Names/Synonyms: C128; DKFZp686B10117; DNA-directed RNA polymerase III 127.6 kDa polypeptide; DNA-directed RNA polymerase III subunit B; DNA-directed RNA polymerase III subunit RPC2; FLJ10388; POLR3B; polymerase (RNA) III (DNA directed) polypeptide B; RNA polymerase III subunit C2; RPC2
Gene Symbols: POLR3B
Molecular weight: 127,785 Da
Basal Isoelectric point: 8.77  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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