Recombinant Human Regulator of nonsense transcripts 3B (UPF3B)

中文名稱：

人UPF3B重組蛋白
貨號：

CSB-YP883646HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人UPF3B重組蛋白
貨號：

CSB-EP883646HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人UPF3B重組蛋白
貨號：

CSB-EP883646HU-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UPF3B重組蛋白
貨號：

CSB-BP883646HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人UPF3B重組蛋白
貨號：

CSB-MP883646HU
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：

UPF3B
Uniprot No.：

Q9BZI7
別名：

UPF3B; RENT3B; UPF3X; Regulator of nonsense transcripts 3B; Nonsense mRNA reducing factor 3B; Up-frameshift suppressor 3 homolog B; hUpf3B; Up-frameshift suppressor 3 homolog on chromosome X; hUpf3p-X
種屬：

Homo sapiens (Human)
蛋白長度：

full length protein
表達區域：

1-483
氨基酸序列

MKEEKEHRPK EKRVTLLTPA GATGSGGGTS GDSSKGEDKQ DRNKEKKEAL SKVVIRRLPP TLTKEQLQEH LQPMPEHDYF EFFSNDTSLY PHMYARAYIN FKNQEDIILF RDRFDGYVFL DNKGQEYPAI VEFAPFQKAA KKKTKKRDTK VGTIDDDPEY RKFLESYATD NEKMTSTPET LLEEIEAKNR ELIAKKTTPL LSFLKNKQRM REEKREERRR REIERKRQRE EERRKWKEEE KRKRKDIEKL KKIDRIPERD KLKDEPKIKV HRFLLQAVNQ KNLLKKPEKG DEKELDKREK AKKLDKENLS DERASGQSCT LPKRSDSELK DEKPKRPEDE SGRDYRERER EYERDQERIL RERERLKRQE EERRRQKERY EKEKTFKRKE EEMKKEKDTL RDKGKKAEST ESIGSSEKTE KKEEVVKRDR IRNKDRPAMQ LYQPGARSRN RLCPPDDSTK SGDSAAERKQ ESGISHRKEG GEE
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：

Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC) and serving as link between the EJC core and NMD machinery. Recruits UPF2 at the cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex (including UPF1 bound to release factors at the stalled ribosome) is believed to activate NMD. In cooperation with UPF2 stimulates both ATPase and RNA helicase activities of UPF1. Binds spliced mRNA upstream of exon-exon junctions. In vitro, stimulates translation; the function is independent of association with UPF2 and components of the EJC core.
基因功能參考文獻：
1. The authors discovered that UPF3B (i) interacts with the release factors, (ii) delays translation termination and (iii) dissociates post-termination ribosomal complexes that are devoid of the nascent peptide. PMID: 28899899
2. UPF3B gene mutation is associated with Lujan-Fryns syndrome. PMID: 26358559
3. the neurodevelopmental phenotype of UPF3B missense mutation is caused by impairment of nonsense-mediated mRNA decay pathway function altering neuronal differentiation. PMID: 26012578
4. These findings indicate that SATB2 activates UPF3B expression through binding to its promoter. PMID: 23925499
5. Data indicate the mutation p.R430X of UPF3B gene as the genetic etiology in the mental retardation pedigree. PMID: 22957832
6. results demonstrate that the UPF3B-dependent NMD pathway is a major regulator of the transcriptome and that its targets have important roles in neuronal cells. PMID: 22182939
7. The two cases with renal dysplasia and developmental delay showed remarkable clinical variability despite having the same mutation in UPF3B. PMID: 22609145
8. Our results demonstrate that in addition to Lujan-Fryns and FG syndromes, UPF3B protein truncation mutations can cause also nonspecific XLMR. PMID: 19238151
9. 3.4 A resolution crystal structure of a minimal UPF3b-EJC assembly, consisting of the interacting domains of five proteins (UPF3b, MAGO, Y14, eIF4AIII, and Barentsz) together with RNA and adenylyl-imidodiphosphate PMID: 20479275
10. A conserved domain of hUpf3b mediates an interaction with the EJC protein Y14. Y14 is required for nonsense-mediated decay induced by tethered hUpf3b. PMID: 12718880
11. The protein region that mediates this interaction and discriminates between hUpf3a and hUpf3b in NMD function is located in the C-terminal domain and fully contained within a small sequence that is highly conserved in Upf3b but not Upf3a proteins PMID: 16601204
12. UPF3B induces nonsense mediated decay in the cytoplasm PMID: 17194930
13. Three mutations lead to the introduction of a premature termination codon and subsequent nonsense-mediated mRNA decay of mutant UPF3B mRNA. PMID: 17704778
14. UPF2 and UPF3b cooperatively stimulate both ATPase and RNA helicase activities of UPF1. PMID: 18066079
15. Results suggest that UPF3A levels are tightly regulated by a post-transcriptional switch to maintain appropriate levels of NMD substrates in cells containing different levels of UPF3B. PMID: 19503078
16. binds to spliced mRNAs upstream of exon-exon junctions; is part of mRNP complexes that are ready for nuclear export and that participate in nonsense-mediated mRNA decay PMID: 11546873
17. binds RNPS1 protein, part of the postsplicing complex deposited 5' to exon-exon junctions PMID: 11546874
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相關疾病：

Mental retardation, X-linked, syndromic, 14 (MRXS14)
亞細胞定位：

Nucleus. Cytoplasm. Note=Shuttling between the nucleus and the cytoplasm.
蛋白家族：

RENT3 family
組織特異性：

Expressed in testis, uterus, prostate, heart, muscle, brain, spinal cord and placenta.
數據庫鏈接：

HGNC: 20439

OMIM: 300298

KEGG: hsa:65109

STRING: 9606.ENSP00000276201

UniGene: Hs.103832