ID:RPC1_HUMAN DESCRIPTION: RecName: Full=DNA-directed RNA polymerase III subunit RPC1; Short=RNA polymerase III subunit C1; EC=2.7.7.6; AltName: Full=DNA-directed RNA polymerase III largest subunit; AltName: Full=DNA-directed RNA polymerase III subunit A; AltName: Full=RNA polymerase III 155 kDa subunit; Short=RPC155; AltName: Full=RNA polymerase III subunit C160; FUNCTION: 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 (By similarity). 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. CATALYTIC ACTIVITY: Nucleoside triphosphate + RNA(n) = diphosphate + RNA(n+1). SUBUNIT: Component of the RNA polymerase III (Pol III) complex consisting of 17 subunits (By similarity). SUBCELLULAR LOCATION: Nucleus. TISSUE SPECIFICITY: Expressed in the brain, in the cortex and the white matter (at protein level). DISEASE: Defects in POLR3A are the cause of leukodystrophy hypomyelinating type 7 with or without oligodontia and/or hypogonadotropic hypogonadism (HLD7) [MIM:607694]. 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. SIMILARITY: Belongs to the RNA polymerase beta' chain family.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
SCOP Domains: 64484 - beta and beta-prime subunits of DNA dependent RNA-polymerase
ModBase Predicted Comparative 3D Structure on O14802
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0002376 immune system process GO:0006351 transcription, DNA-templated GO:0006383 transcription from RNA polymerase III promoter GO:0032728 positive regulation of interferon-beta production GO:0045087 innate immune response GO:0051607 defense response to virus
BC014399 - Homo sapiens polymerase (RNA) III (DNA directed) polypeptide A, 155kDa, mRNA (cDNA clone IMAGE:3356934), complete cds. AK024744 - Homo sapiens cDNA: FLJ21091 fis, clone CAS03642. JD112287 - Sequence 93311 from Patent EP1572962. JD216160 - Sequence 197184 from Patent EP1572962. BC041089 - Homo sapiens polymerase (RNA) III (DNA directed) polypeptide A, 155kDa, mRNA (cDNA clone MGC:45186 IMAGE:3915501), complete cds. AK300329 - Homo sapiens cDNA FLJ61707 complete cds, highly similar to DNA-directed RNA polymerase III largest subunit (EC 2.7.7.6). AK026023 - Homo sapiens cDNA: FLJ22370 fis, clone HRC06677, highly similar to AF021351 Homo sapiens RNA polymerase III largest subunit (hRPC155) mRNA. AY091462 - Homo sapiens RNA polymerase III subunit RPC155-D mRNA, partial cds. AF021351 - Homo sapiens RNA polymerase III largest subunit (hRPC155) mRNA, complete cds. AB384941 - Synthetic construct DNA, clone: pF1KB4393, Homo sapiens POLR3A gene for DNA-directed RNA polymerase III subunit RPC1, complete cds, without stop codon, in Flexi system. AY091461 - Homo sapiens RNA polymerase III subunit RPC155-C mRNA, partial cds. AY091460 - Homo sapiens RNA polymerase III subunit RPC155-B mRNA, partial cds. AY091459 - Homo sapiens RNA polymerase III subunit RPC155-A mRNA, partial cds.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein O14802 (Reactome details) participates in the following event(s):
R-HSA-76056 Recruitment of RNA polymerase III to TFIIIB:TFIIIC:TFIIIA:Type 1 Promoter Complex R-HSA-83803 Recruitment of RNA Polymerase III to TFIIIB:SNAPc:Type 3 Promoter Complex R-HSA-83805 Recruitment of RNA Polymerase III to the TFIIIB:TFIIIC: Type 2 Promoter Complex R-HSA-113454 RNA Polymerase III Termination and release of transcribed RNA R-HSA-76060 RNA Polymerase III Promoter Opening at Type 1 Promoters R-HSA-112150 RNA Polymerase III Promoter Opening at Type 2 Promoters R-HSA-112152 RNA Polymerase III Promoter Opening at Type 3 Promoters R-HSA-112055 RNA Polymerase III Abortive Initiation At Type 1 Open Promoters R-HSA-112153 RNA Polymerase III Simple Start Sequence Initiation At Type 1 Promoters R-HSA-112149 RNA Polymerase III Abortive Initiation At Type 2 Open Promoters R-HSA-112155 RNA Polymerase III Simple Start Sequence Initiation At Type 2 Promoters R-HSA-112054 RNA Polymerase III Abortive Initiation At Type 3 Open Promoters R-HSA-112156 RNA Polymerase III Simple Start Sequence Initiation At Type 3 Promoters R-HSA-113442 RNA Polymerase III Retractive RNase Activity at U-tract Pause Sites R-HSA-113446 Initiation of RNA Polymerase III Productive Transcription R-HSA-113449 RNA Polymerase III Transcriptional Pause at Terminator Sequence R-HSA-113451 Resumption of RNA Polymerase III Productive Transcription R-HSA-113705 RNA Polymerase III Productive Transcription R-HSA-76061 RNA Polymerase III Transcription Initiation From Type 1 Promoter R-HSA-76071 RNA Polymerase III Transcription Initiation From Type 3 Promoter R-HSA-76066 RNA Polymerase III Transcription Initiation From Type 2 Promoter R-HSA-73980 RNA Polymerase III Transcription Termination R-HSA-749476 RNA Polymerase III Abortive And Retractive Initiation R-HSA-73780 RNA Polymerase III Chain Elongation R-HSA-76046 RNA Polymerase III Transcription Initiation R-HSA-74158 RNA Polymerase III Transcription R-HSA-1834949 Cytosolic sensors of pathogen-associated DNA R-HSA-74160 Gene expression (Transcription) R-HSA-168249 Innate Immune System R-HSA-168256 Immune System