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  4. PNPT1 Antibody

PNPT1 Antibody

  • 货号：
    CSB-PA368444
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of 293 cells using PNPase Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    Q8TCS8
  • 基因名：
    PNPT1
  • 别名：
    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
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Synthesized peptide derived from the C-terminal region of Human PNPase.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:20000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Combined oxidative phosphorylation deficiency 13 (COXPD13); Deafness, autosomal recessive, 70 (DFNB70)
  • 亚细胞定位：
    Cytoplasm. Mitochondrion matrix. Mitochondrion intermembrane space; Peripheral membrane protein.
  • 蛋白家族：
    Polyribonucleotide nucleotidyltransferase family
  • 数据库链接：

    HGNC: 23166

    OMIM: 610316

    KEGG: hsa:87178

    STRING: 9606.ENSP00000393953

    UniGene: Hs.388733