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PRPF8 Antibody

  • 货号：
    CSB-PA744036LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunofluorescence staining of Hela cells with CSB-PA744036LA01HU at 1:166, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PRPF8 Polyclonal antibody
  • Uniprot No.：
    Q6P2Q9
  • 基因名：
    PRPF8
  • 别名：
    220 kDa U5 snRNP specific protein antibody; 220 kDa U5 snRNP-specific protein antibody; Apoptosis regulated protein 1 antibody; Apoptosis regulated protein 2 antibody; HPRP8 antibody; p220 antibody; Pre mRNA processing factor 8 antibody; Pre mRNA-processing factor 8; S. cerevisiae; homolog of antibody; Pre-mRNA-processing-splicing factor 8 antibody; Precursor mRNA processing protein antibody; PRP8 antibody; PRP8 homolog antibody; PRP8 pre mRNA processing factor 8 homolog antibody; PRP8_HUMAN antibody; PRPC8 antibody; Prpf8 antibody; Retinitis pigmentosa 13 (autosomal dominant) antibody; RP13 antibody; SNRNP220 antibody; Splicing factor Prp8 antibody; U5 snRNP specific protein antibody; U5 snRNP specific protein (220 kD); ortholog of S. cerevisiae Prp8p antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Pre-mRNA-processing-splicing factor 8 protein (2037-2170AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PRPF8 Antibody (CSB-PA744036LA01HU)，的标记方式是Non-conjugated。对于PRPF8 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA744036LB01HU PRPF8 Antibody, HRP conjugated ELISA
    FITC CSB-PA744036LC01HU PRPF8 Antibody, FITC conjugated
    Biotin CSB-PA744036LD01HU PRPF8 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:50-1:200
  • 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 core component of precatalytic, catalytic and postcatalytic spliceosomal complexes, both of the predominant U2-type spliceosome and the minor U12-type spliceosome. Functions as a scaffold that mediates the ordered assembly of spliceosomal proteins and snRNAs. Required for the assembly of the U4/U6-U5 tri-snRNP complex, a building block of the spliceosome. Functions as scaffold that positions spliceosomal U2, U5 and U6 snRNAs at splice sites on pre-mRNA substrates, so that splicing can occur. Interacts with both the 5' and the 3' splice site.
  • 基因功能参考文献：
    1. Multiple genes contributing to the retinal dystrophy genotypes within a family were discovered using retinal gene-targeted next-generation sequencing. Families with noted examples of phenotypic variation or apparent non-penetrant individuals may offer a clue to suspect complex inheritance. PMID: 28761320
    2. Frame-shift mutations and nonconservative amino acid changes in PRPF8 typically cause severe clinical phenotypes. The conservative missense variant p.PRPF8-Arg2310Lys that is not altering the global charge of the C-terminal tail, and variants located at the basis of the C-terminal tail show milder clinical phenotypes, in accordance with functional data on PRPF8/SNRNP200 interactions in yeast. PMID: 29087248
    3. HSP90/R2TP chaperone system promotes the assembly of a key module of U5 snRNP while assuring the quality control of PRPF8. The proteomics data further reveal new interactions between R2TP and the tuberous sclerosis complex, pointing to a potential link between growth signals and the assembly of key cellular machines. PMID: 28515276
    4. influenza A virus upregulates cellular PRPF8 gene expression through viral NS1 protein and influenza virus polymerase basic protein 1 to increase virus production. PMID: 28110426
    5. Our findings exemplify the regulatory potential of changes in the core spliceosome machinery, which may be relevant to slow-onset human genetic diseases linked to PRPF8 deficiency PMID: 26392272
    6. Most importantly between Prp8 and nucleotides at the exon-intron junction. PMID: 26385511
    7. A mutation in a splicing factor PRPF8 that causes retinitis pigmentosa has a transcriptome-wide effect on mRNA splicing. PMID: 24969741
    8. Data suggest Enterovirus 3DPol (RNA-dependent RNA polymerase) enters nucleus via nuclear localization signal, targets pre-mRNA processing factor 8 (Prp8) to block pre-mRNA splicing/mRNA synthesis, and shuts off cellular transcription/translation. PMID: 24968230
    9. In the cytoplasm, Prp8 forms a precursor complex with U5 snRNA PMID: 23727230
    10. these data show how a Ski2-like RNA helicase Brr2 can be reversibly inhibited by a protein cofactor Prp8 that directly competes with RNA substrate binding. PMID: 23704370
    11. This is the first report of marked intrafamilial variability associated with mutations in the PRPF8 gene, including incomplete penetrance. PRPF8 mutations should be suspected in patients with autosomal dominant retinitis pigmentosa. PMID: 22039234
    12. RP-PRPF defects affect the stoichiometry of spliceosomal small nuclear RNAs. Depleting PRPF8 in human cell lines alters alternative splicing. PMID: 21378395
    13. Data screened retinitis pigmentosa patients for PRPF8 mutations and identified three new missense mutations, including the first documented mutation outside exon 42 and the first de novo mutation. PMID: 20232351
    14. Mutations in PRPC8 is associated with autosomal dominant retinitis pigmentosa PMID: 11910553
    15. mutations revealed a novel insertion and deletion in the last exon of a splicing factor gene, PRPF8. PMID: 12601059
    16. 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
    17. The gene for apoptosis regulated protein 2 (ARP2) overexpressed in apoptosis induced prostate cancer cell line LNCaP was cloned. PMID: 15786732
    18. The PRPF8 gene mutation is associated with a mild phenotype in which cone function is partially preserved. PMID: 17061239
    19. The expanded Prp8 Jab1/MPN domain represents a pseudoenzyme converted into a protein-protein interaction platform and that dysfunction of this platform underlies Retinitis pigmentosa. PMID: 17317632
    20. The severe form of autosomal dominant retinitis pigmentosa (adRP) was caused by the PRPF8 H2309R variant, whereas the IVS41-4G-->A variant was benign. PMID: 18695108
    21. Crystal structures of corresponding portions of yeast and human Prp8 that interact with functional regions of the pre-mRNA were determined, revealing a phylogenetically conserved RNase H fold, augmented by Prp8-specific elements. PMID: 18843295
    22. The aim of this study was to use lymphoblast cell lines derived from retinitis pigmentosa patients to determine whether mutations in two of these splicing factors, PRPF8 and PRPF31, cause measurable deficiencies in pre-mRNA splicing. PMID: 19096719

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  • 相关疾病：
    Retinitis pigmentosa 13 (RP13)
  • 亚细胞定位：
    Nucleus. Nucleus speckle.
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 17340

    OMIM: 600059

    KEGG: hsa:10594

    STRING: 9606.ENSP00000304350

    UniGene: Hs.181368