PNPT1 Antibody

說明書

中文名稱：

PNPT1兔多克隆抗體
貨號：

CSB-PA796358
規格：

￥1100
圖片：
The image on the left is immunohistochemistry of paraffin-embedded Human gastic cancer tissue using CSB-PA796358(PNPT1 Antibody) at dilution 1/40, on the right is treated with synthetic peptide. (Original magnification: ×200)

The image on the left is immunohistochemistry of paraffin-embedded Human prostate cancer tissue using CSB-PA796358(PNPT1 Antibody) at dilution 1/40, on the right is treated with synthetic peptide. (Original magnification: ×200)

Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-5: Jurkat cells, Hela cells, A431 cells, mouse liver tissue tissue, 293T cells, Primary antibody: CSB-PA796358(PNPT1 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 2 minutes
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q8TCS8
基因名：

PNPT1
別名：

PNPT1 antibody; PNPASE antibody; Polyribonucleotide nucleotidyltransferase 1 antibody; mitochondrial antibody; EC 2.7.7.8 antibody; 3'-5' RNA exonuclease OLD35 antibody; PNPase old-35 antibody; Polynucleotide phosphorylase 1 antibody; PNPase 1 antibody; Polynucleotide phosphorylase-like protein antibody
宿主：

Rabbit
反應種屬：

Human,Mouse
免疫原：

Synthetic peptide of Human PNPT1
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

Antigen affinity purification
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC

推薦稀釋比：

Application	Recommended Dilution
ELISA	1:1000-1:2000
WB	1:200-1:1000
IHC	1:50-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

RNA-binding protein implicated in numerous RNA metabolic processes. Catalyzes the phosphorolysis of single-stranded polyribonucleotides processively in the 3'-to-5' direction. Mitochondrial intermembrane factor with RNA-processing exoribonulease activity. Component of the mitochondrial degradosome (mtEXO) complex, that degrades 3' overhang double-stranded RNA with a 3'-to-5' directionality in an ATP-dependent manner. Involved in the degradation of non-coding mitochondrial transcripts (MT-ncRNA) and tRNA-like molecules. Required for correct processing and polyadenylation of mitochondrial mRNAs. Plays a role as a cytoplasmic RNA import factor that mediates the translocation of small RNA components, like the 5S RNA, the RNA subunit of ribonuclease P and the mitochondrial RNA-processing (MRP) RNA, into the mitochondrial matrix. Plays a role in mitochondrial morphogenesis and respiration; regulates the expression of the electron transport chain (ETC) components at the mRNA and protein levels. In the cytoplasm, shows a 3'-to-5' exoribonuclease mediating mRNA degradation activity; degrades c-myc mRNA upon treatment with IFNB1/IFN-beta, resulting in a growth arrest in melanoma cells. Regulates the stability of specific mature miRNAs in melanoma cells; specifically and selectively degrades miR-221, preferentially. Plays also a role in RNA cell surveillance by cleaning up oxidized RNAs. Binds to the RNA subunit of ribonuclease P, MRP RNA and miR-221 microRNA.
基因功能參考文獻：
1. The PNPT1 p.Arg136His and p.Pro140Leu variants in our subject showed 1) the mutated amino acids are highly conserved and our structural analysis supported them to be functionally deleterious; 2) The carrier frequencies of the variants are very low in populations (1:60.000), with no homozygous carriers found; 3) The RC complex amounts in the subject's myoblasts were functionally rescued by expression of wild-type PNPT1. PMID: 28645153
2. Inhibition of homologous PNPase by citrate may represent an evolutionarily conserved communicative link between RNA degradation and central metabolism. PMID: 28334892
3. In vitro rescue experiments, using exogenous expression of wild-type PNPT1 in patient fibroblasts, ameliorated the deficiencies in the OXPHOS complex protein expression, supporting the likely pathogenicity of these variants and the importance of Whole-exome sequencingin efficiently identifying rare genetic disease genes PMID: 27759031
4. this study provides further evidence that hPNPase(old-35) is associated with global changes in cell cycle-associated genes and identifies potential gene targets for future investigation PMID: 24729470
5. fresh insight into cellular pathways regulated by PNPT1 PMID: 24143183
6. Interaction between PNPase and hSuv3 is essential for efficient mitochondrial RNA degradation. PMID: 23221631
7. A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss. PMID: 23084290
8. Mutation in PNPT1, which encodes a polyribonucleotide nucleotidyltransferase, impairs RNA import into mitochondria and causes respiratory-chain deficiency. PMID: 23084291
9. a novel role of nEGFR in radioresistance, and that is, upon ionizing radiation, nEGFR inactivates the ribonuclease activity of PNPase toward c-MYC mRNA through DNAPK-mediated Ser-776 phosphorylation PMID: 22815474
10. The study provides structural and functional insights into hPNPase, which uses a KH pore to trap a long RNA 3' tail that is further delivered into an RNase PH channel for the degradation process. PMID: 22210891
11. targeted overexpression of hPNPase(old-35) represents a novel strategy to selectively downregulate RNA expression and consequently intervene in a variety of pathophysiological conditions PMID: 21151174
12. The data support an unanticipated role for PNPASE in mediating the translocation of RNAs into mitochondria. PMID: 20691904
13. Inhibition of PNPase by shRNA or stable overexpression of miR-221 protected melanoma cells from IFN-beta-mediated growth inhibition, accentuating the importance of PNPase induction and miR-221 down-regulation in mediating IFN-beta action. PMID: 20547861
14. Protein-protein interactions between human exosome components support the assembly of RNase PH-type subunits into a six-membered PNPase-like ring PMID: 12419256
15. an important role for hPNPase(old-35)in growth control associated with terminal differentiation and cellular senescence. PMID: 12473748
16. the molecular mechanism of the growth-arresting property of hPNPaseold-35 PMID: 12721301
17. demonstrate that the hPNPase is localized in mitochondria; finding suggests the involvement of mitochondrial RNA metabolism in cellular senescence PMID: 12798676
18. analysis of domains of human polynucleotide phosphorylase (hPNPaseOLD-35) mediating cellular senescence PMID: 16055741
19. Targeted overexpression of hPNPase(old-35) might be a novel therapeutic strategy for c-myc-overexpressing and IFN-resistant tumors, such as melanomas. PMID: 16410805
20. human PNPase mRNA upregulation by beta-interferon has no effect on protein level in melanoma cell lines PMID: 16505900
21. PNPase localization to the mitochondrial intermembrane space suggests a unique role distinct from its highly conserved function in RNA processing in chloroplasts and bacteria. PMID: 16966379
22. Combined, the data demonstrate an unexpected IMS localization and a key role for PNPase in maintaining mitochondrial homeostasis. PMID: 16966381
23. A novel pathway by which an evolutionary conserved RNA-metabolizing enzyme, hPNPase(old-35), regulates cell growth and viability. PMID: 17804700
24. recent advances in understanding the various roles of hPNPase both within and potentially outside of the mitochondria [review] PMID: 17983748
25. In this work, the degradation, polymerization, and RNA-binding properties of the human PNPase were analyzed and compared to its bacterial and organellar counterparts. PMID: 18083836
26. Involvement of human polynucleotide phosphorylase in mtRNA degradation was studied. PMID: 18083837
27. The complex of hSUV3-hPNPase is an integral entity for efficient degradation of structured RNA and may be the long sought RNA-degrading complex in the mammalian mitochondria. PMID: 19509288
28. maintained expression and even up-regulation of some (PNPT1, PMPCB, HMMR/RHAMM, BSG and ERCC1) tumor associated antigens in CD40-activated leukemic cells. PMID: 19580345
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相關疾病：

Combined oxidative phosphorylation deficiency 13 (COXPD13); Deafness, autosomal recessive, 70 (DFNB70)
亞細胞定位：

Cytoplasm. Mitochondrion matrix. Mitochondrion intermembrane space; Peripheral membrane protein.
蛋白家族：

Polyribonucleotide nucleotidyltransferase family
數據庫鏈接：

HGNC: 23166

OMIM: 610316

KEGG: hsa:87178

STRING: 9606.ENSP00000393953

UniGene: Hs.388733

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