Recombinant Human DNA-directed RNA polymerase III subunit RPC2 (POLR3B), partial

中文名稱：

人POLR3B重組蛋白
貨號：

CSB-YP885742HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人POLR3B重組蛋白
貨號：

CSB-EP885742HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人POLR3B重組蛋白
貨號：

CSB-EP885742HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

人POLR3B重組蛋白
貨號：

CSB-BP885742HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人POLR3B重組蛋白
貨號：

CSB-MP885742HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

POLR3B
Uniprot No.：

Q9NW08
別名：

C128; DNA directed RNA polymerase III 127.6 kDa polypeptide; DNA directed RNA polymerase III subunit B; 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; RNA polymerase III subunit RPC2; RPC2; RPC2_HUMAN
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. Novel compound heterozygous variations in POLR3B were identified in a patient with cerebellar hypoplasia with endosteal sclerosis. PMID: 28589944
2. The spectrum of phenotypes resulting from POLR3B mutations is wider than previously believed. PMID: 27512013
3. 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
4. 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
5. first reports of long deletions causing POLR3-related leukodystrophy, suggesting that deletions and duplications in POLR3A or POLR3B should be investigated in patients with a compatible phenotype PMID: 26045207
6. Mutations in POLR3A or POLR3B are rare in patients with unclassified hypomyelination. PMID: 26011300
7. INMAP as a model regulator of CENP-B PMID: 24633075
8. These results suggest that INMAP might function through p53/p21 pathways. PMID: 25635878
9. Most patients with 4H leukodystrophy carried the common c.1568T>A POLR3B mutation on one allele. PMID: 25339210
10. 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
11. 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
12. study reports INMAP is a truncated version of POLR3B, and is up-regulated in several human cancer cell lines; results suggest that INMAP may function through the p53 and AP-1 pathways, providing a possible link of its activity with tumourigenesis PMID: 23124897
13. Mutations in POLR3A and POLR3B encoding RNA Polymerase III subunits cause an autosomal-recessive hypomyelinating leukoencephalopathy PMID: 22036171
14. Recessive mutations in POLR3B, encoding the second largest subunit of Pol III, cause a rare hypomyelinating leukodystrophy PMID: 22036172
15. Results suggest that INMAP is a novel protein that plays essential role in spindle formation and cell-cycle progression. PMID: 19331820
16. Data describe the purification and identification of RNA polymerase III subunits RPC2 and RPC5. PMID: 12391170
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相關疾病：

Leukodystrophy, hypomyelinating, 8, with or without oligodontia and/or hypogonadotropic hypogonadism (HLD8)
亞細胞定位：

Nucleus.
蛋白家族：

RNA polymerase beta chain family
數據庫鏈接：

HGNC: 30348

OMIM: 614366

KEGG: hsa:55703

STRING: 9606.ENSP00000228347

UniGene: Hs.610795