POLR3A Antibody

Code CSB-PA018343LA01HU
Size US$299Purchase it in Cusabio online store
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  • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA018343LA01HU at dilution of 1:100

  • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA018343LA01HU at dilution of 1:100

  • Chromatin Immunoprecipitation 293T (1.6*106) were cross-linked with formaldehyde, sonicated, and immunoprecipitated with 4µg anti-POLR3A or a control normal rabbit IgG. The resulting ChIP DNA was quantified using real-time PCR with primers (CSB-PP018343HU) against the tRNA-Leu Anti-Codon (TAG) promoter.

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Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) POLR3A Polyclonal antibody
Uniprot No. O14802
Target Names POLR3A
Alternative Names BC053071 antibody; DNA directed RNA polymerase III largest subunit antibody; DNA directed RNA polymerase III subunit A antibody; DNA-directed RNA polymerase III largest subunit antibody; DNA-directed RNA polymerase III subunit A antibody; DNA-directed RNA polymerase III subunit RPC1 antibody; hRPC155 antibody; MGC62420 antibody; POLR 3A antibody; POLR3A antibody; Polymerase (RNA) III (DNA directed) polypeptide A 155kDa antibody; Polymerase (RNA) III (DNA directed) polypeptide A antibody; RGD1305574 antibody; RNA polymerase III 155 kDa subunit antibody; RNA polymerase III subunit C1 antibody; RNA polymerase III subunit C160 antibody; RNA polymerase III subunit RPC155 D antibody; RPC1 antibody; RPC1_HUMAN antibody; RPC155 antibody
Raised in Rabbit
Species Reactivity Human
Immunogen Recombinant Human DNA-directed RNA polymerase III subunit RPC1 protein (392-632AA)
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Clonality Polyclonal
Isotype IgG
Purification Method >95%, Protein G purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form Liquid
Tested Applications ELISA, IHC, ChIP
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
Protocols ELISA Protocol
Immunohistochemistry (IHC) Protocol
Native Chromatin Immunoprecipitation(ChIP) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Target Data

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.
Gene References into Functions
  1. we firmly establish biallelic mutations in POLR3A as the genetic cause of a recognizable, neonatal, Wiedemann-Rautenstrauch-like progeroid syndrome. Thus, we suggest that POLR3A mutations are causal for a portion of under-diagnosed early-onset segmental progeroid syndromes PMID: 30450527
  2. RNA polymerase III (Pol III) transcribes medium-sized non-coding RNAs (collectively termed Pol III genes), and data show that when Pol III genes are hypo-methylated, MYC amplifies their transcription, regardless of its recognition DNA motif. PMID: 28846112
  3. the first transgenic mice with a leukodystrophy-causing Polr3a mutation do not recapitulate the childhood-onset hypomyelinating leukodystrophy observed in the majority of human patients with POLR3A mutations. PMID: 28407788
  4. This is the first individual reported with two truncating POLR3A variants, suggesting that biallelic severe loss of function variants are associated with WRS. Sequencing of POLR3A and perhaps related genes such as POLR3B in additional patients with clinical findings of WRS is needed to prove this gene-disease association. PMID: 27612211
  5. Findings suggest that AP-1 factors are regulators of RNA polymerase III (Pol III)-driven 5S rRNA and U6 snRNA expression with a potential role in cell proliferation. PMID: 28488757
  6. For some of its complex functions, variation in RNAP III activity levels lead to nonuniform changes in tRNA pools that can shift the translation profiles of key codon-biased mRNAs with resultant phenotypes or disease states. (Review) PMID: 27068803
  7. Mutations in POLR3A are a frequent cause of sporadic and recessive spastic ataxia. PMID: 28459997
  8. RNA polymerase III (RNAPIII) is specialized for transcription of short, abundant nonprotein-coding RNA transcripts. PMID: 27911719
  9. Our transcriptome-wide investigations revealed an overall decrease in the levels of Pol III-transcribed tRNAs and an imbalance in the levels of regulatory ncRNAs such as small nuclear and nucleolar RNAs (snRNAs and snoRNAs). PMID: 27506977
  10. Multicenter retrospective study to collect neuroradiologic, clinical, and molecular data of patients with mutations in POLR3A and POLR3B without the classic MRI phenotype: diffuse hypomyelination is not an obligatory feature of POLR3-related disorders; two distinct patterns, selective involvement of the corticospinal tracts and cerebellar atrophy, are added to the MRI presentation of POLR3-related disorders PMID: 27029625
  11. Mutations in POLR3A or POLR3B are rare in patients with unclassified hypomyelination. PMID: 26011300
  12. Mutations in POLR3A are associated with a more severe clinical course of 4H leukodystrophy. PMID: 25339210
  13. MRI in patients with POLR3B mutations revealed smaller cerebellar structures, especially vermis, than those in POLR3A mutations. MRI also showed milder hypomyelination in patients with POLR3B mutations than those with POLR3A mutations PMID: 23643445
  14. Investigated POLR3A and POLR3B mutations in patients with genetically unexplained hypomyelinating leukodystrophies with features of Pol III-related leukodystrophies. Recessive mutations in POLR3A or POLR3B were uncovered in all 14 patients. PMID: 23355746
  15. Studies indicate that aatients with anti-RNAP have an increased risk of malignancy within a 5-year timeframe before or after onset of systemic sclerosis (SSc) skin changes. PMID: 22189167
  16. Mutations in POLR3A and POLR3B encoding RNA Polymerase III subunits cause an autosomal-recessive hypomyelinating leukoencephalopathy PMID: 22036171
  17. Mutations of POLR3A encoding a catalytic subunit of RNA polymerase Pol III cause a recessive hypomyelinating leukodystrophy. PMID: 21855841

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Involvement in disease Leukodystrophy, hypomyelinating, 7, with or without oligodontia and/or hypogonadotropic hypogonadism (HLD7)
Subcellular Location Nucleus
Protein Families RNA polymerase beta' chain family
Tissue Specificity Expressed in the brain, in the cortex and the white matter (at protein level).
Database Links

HGNC: 30074

OMIM: 607694

KEGG: hsa:11128

STRING: 9606.ENSP00000361446

UniGene: Hs.436896

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