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PRPF3 Antibody

  • 货号：
    CSB-PA018763GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    O43395
  • 基因名：
    PRPF3
  • 别名：
    hPrp3 antibody; HPRP3P antibody; Pre mRNA splicing factor 3 antibody; Pre-mRNA-splicing factor 3 antibody; Precursor mRNA-processing factor 3; S. crevisiae; homolog of antibody; PRP3 pre-mRNA processing factor 3 homolog (S. cerevisiae) antibody; Prp3p antibody; PRPF3 antibody; PRPF3_HUMAN antibody; RP18 antibody; SNRNP90 antibody; U4/U6 small nuclear ribonucleoprotein PRP3 antibody; U4/U6 snRNP 90 kDa protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PRPF3
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 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：
    Plays role in pre-mRNA splicing as component of the U4/U6-U5 tri-snRNP complex that is involved in spliceosome assembly, and as component of the precatalytic spliceosome (spliceosome B complex).
  • 基因功能参考文献：
    1. Results from whole-exome sequencing identified 2 variants c.1345C > G (p.R449G) and c.1532A > C (p.H511P) in PRPF3 which co-segregate with retinitis pigmentosa in two families respectively. PMID: 27886254
    2. SUMO conjugation plays a role during mRNA splicing processes including a role for Prp3 SUMOylation in U4/U6*U5 tri-snRNP formation and/or recruitment. PMID: 28379520
    3. RP-PRPF defects affect the stoichiometry of spliceosomal small nuclear RNAs. Mutant PRPF3 proteins stably associated with tri-snRNPs. PMID: 21378395
    4. A mutation in the PRPF3 gene is rare compared to other genes causing autosomal dominant retinitis pigmentosa. PMID: 20309403
    5. Mutations in HPRP3, a third member of pre-mRNA splicing factor genes, implicated in autosomal dominant retinitis pigmentosa. PMID: 11773002
    6. role in the recruitment of Hprp4p for the U4/U6 snRNP assembly PMID: 11971898
    7. Nine mutations, six of which are novel, in the pre-mRNA splicing-factor genes PRPF3, PRPF8, and PRPF31, causing adRP have been identified in the Spanish population. PMID: 12714658
    8. Free and complexed cyclophilin H have virtually identical conformations suggesting that the U4/U6-60K binding site is pre-shaped and the peptidyl-prolyl-cis/trans isomerase activity is unaffected by complex formation PMID: 12875835
    9. We conclude that the Thr494Met mutation in the HPRP3 gene causes ADRP in Japanese patients. This mutation was found in 1% of patients with ADRP in Japan PMID: 15085354
    10. PAP-1 interacted with Prp3p but not Prp31p in human cells and yeast, and the basic region of PAP-1 and the C-terminal region of Prp3p, regions beside spots found in retinitis pigmentosa mutations, were needed for binding PMID: 15541726
    11. splicing factor PRPF3 mutations cause retinal degeneration and form detrimental aggregates in photoreceptor cells PMID: 17517693
    12. Findings suggest that the loss of Hprp3p phosphorylation at Thr494 is a key step for initiating Thr494Met aberrant interactions within U4/U6 snRNP complex and these are likely linked to the retinitis pigmentosa type 18 phenotype. PMID: 17932117
    13. splicing activity is significantly influenced by the CK2-hPrp3p interaction PMID: 18026141
    14. TASP1, EPS15R, and PRPF3 expression were significantly induced in HCCs of transgenic EGF2B mice as was P2 promoter-driven HNF4alpha PMID: 18395097
    15. These data support the notion about individual roles for CK2alpha and CK2alpha' in the splicing process. PMID: 18553058
    16. The human HPRP3 gene, the orthologue of the yeast pre-mRNA splicing factor (PRP3) PMID: 11773002

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  • 相关疾病：
    Retinitis pigmentosa 18 (RP18)
  • 亚细胞定位：
    Nucleus. Nucleus speckle.
  • 组织特异性：
    Highly expressed in retina, liver, kidney and blood. Detected at lower levels in heart and brain.
  • 数据库链接：

    HGNC: 17348

    OMIM: 601414

    KEGG: hsa:9129

    STRING: 9606.ENSP00000315379

    UniGene: Hs.11776