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Recombinant Human U4/U6 small nuclear ribonucleoprotein Prp31 (PRPF31)

  • 中文名稱:
    人PRPF31重組蛋白
  • 貨號:
    CSB-YP823900HU
  • 說明書:
  • 規格:
  • 來源:
    Yeast
  • 其他:
  • 中文名稱:
    人PRPF31重組蛋白
  • 貨號:
    CSB-EP823900HU
  • 說明書:
  • 規格:
  • 來源:
    E.coli
  • 其他:
  • 中文名稱:
    人PRPF31重組蛋白
  • 貨號:
    CSB-EP823900HU-B
  • 說明書:
  • 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PRPF31重組蛋白
  • 貨號:
    CSB-BP823900HU
  • 說明書:
  • 規格:
  • 來源:
    Baculovirus
  • 其他:
  • 中文名稱:
    人PRPF31重組蛋白
  • 貨號:
    CSB-MP823900HU
  • 說明書:
  • 規格:
  • 來源:
    Mammalian cell
  • 其他:

產品詳情

  • 純度:
    >85% (SDS-PAGE)
  • 基因名:
    PRPF31
  • Uniprot No.:
  • 別名:
    DKFZp566J153; hPrp 31; hPrp31; NY BR 99; Pre mRNA processing factor 31; Pre mRNA processing factor 31 homolog (yeast); Pre mRNA processing factor 31 homolog; Pre-mRNA-processing factor 31; Precursor mRNA-processing factor 31; S. cerevisiae; homolog of; Protein 61K; PRP 31; PRP31; PRP31 pre mRNA processing factor 31 homolog (yeast); PRP31 pre mRNA processing factor 31 homolog; PRP31_HUMAN; PRPF 31; prpf31; RP 11; RP11; Serologically defined breast cancer antigen NY BR 99; Serologically defined breast cancer antigen NY-BR-99; SNRNP61; U4/U6 small nuclear ribonucleoprotein Prp31; U4/U6 snRNP 61 kDa protein
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    full length protein
  • 表達區域:
    1-499
  • 氨基酸序列
    MSLADELLAD LEEAAEEEEG GSYGEEEEEP AIEDVQEETQ LDLSGDSVKT IAKLWDSKMF AEIMMKIEEY ISKQAKASEV MGPVEAAPEY RVIVDANNLT VEIENELNII HKFIRDKYSK RFPELESLVP NALDYIRTVK ELGNSLDKCK NNENLQQILT NATIMVVSVT ASTTQGQQLS EEELERLEEA CDMALELNAS KHRIYEYVES RMSFIAPNLS IIIGASTAAK IMGVAGGLTN LSKMPACNIM LLGAQRKTLS GFSSTSVLPH TGYIYHSDIV QSLPPDLRRK AARLVAAKCT LAARVDSFHE STEGKVGYEL KDEIERKFDK WQEPPPVKQV KPLPAPLDGQ RKKRGGRRYR KMKERLGLTE IRKQANRMSF GEIEEDAYQE DLGFSLGHLG KSGSGRVRQT QVNEATKARI SKTLQRTLQK QSVVYGGKST IRDRSSGTAS SVAFTPLQGL EIVNPQAAEK KVAEANQKYF SSMAEFLKVK GEKSGLMST
  • 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 Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
    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

相關產品

靶點詳情

  • 功能:
    Involved in pre-mRNA splicing as component of the spliceosome. Required for the assembly of the U4/U5/U6 tri-snRNP complex, one of the building blocks of the spliceosome.
  • 基因功能參考文獻:
    1. We have identified a previously unreported missense mutation of PRPF31 that resulted in incomplete penetrance in a family with night blindness a Retinitis pigmentosa. PMID: 30099644
    2. A novel heterozygous insertion frameshift mutation c.1226_1227insA, p.T410Dfs*65, and a novel heterozygous stopgain mutation c.1015C>T, p.Q339* were identified in PRPF31. PMID: 29957067
    3. Common variants in PRPF31 and CPA6 were associated with worse and better metformin response, respectively. PMID: 29650774
    4. Mutation analysis revealed PRPF31 mutations as the cause for autosomal dominant RP in both patients PMID: 29305715
    5. we identified the biological function of lncRNA RP11-838N2.4 in temolozomide resistance in glioblastoma PMID: 27270310
    6. Our study revealed novel mutations of PRPF31 in RP. Our results also showed that the two mutations (c.547delG or c.804delG) affect gene expression and GFP-PRPF31sg has increased protein stability. PMID: 29260190
    7. We also demonstrate that across the human genome, the presence of MSR1 repeats in the promoters or first introns of genes is associated with greater population variability in gene expression indicating that copy number variation of MSR1s is a generic controller of gene expression and promises to provide new insights into our understanding of gene expression regulation PMID: 26781568
    8. In view of the high genetic heterogeneity of PRPF31 mutations, the screening must include the entire gene, as well as CNV assays, to detect large rearrangements. PMID: 28192796
    9. This study identified host factor PRPF31 as a cellular factor involved in Hepatitis B virus covalently closed circular DNA (cccDNA)formation. PRPF31 is recruited to cccDNA. Chromatin immunoprecipitation and immunoprecipitation assays revealed an association between PRPF31 and cccDNA. PMID: 27864147
    10. Variant haploinsufficiency and phenotypic non-penetrance in PRPF31-associated retinitis pigmentosa has been described. PMID: 26853529
    11. This is the first Swedish report of the clinical phenotype associated with a mutation in the PRPF31 (p.IVS6+1G>T) gene. PMID: 27212874
    12. The small nuclear ribonucleoprotein U4 core-domain structure has now been re-refined. The U4 Sm site-sequence AAUUUUU has been shown to bind to the seven Sm proteins SmF-SmE-SmG-SmD3-SmB-SmD1-SmD2 in an identical manner as the U1 Sm-site sequence AAUUUGU, except in SmD1 where the bound U replaces G. PMID: 26894541
    13. As PRPF31 mutations cause retinitis pigmentosa by haploinsufficiency, this is a good candidate for adeno-associated virus (AAV)-based gene augmentation therapy. PMID: 26959129
    14. A PRPF31 mutation was identified to be responsible for adRP in a large Chinese family. Our findings expand the mutation spectrum of RP in the Chinese population. PMID: 23834559
    15. A mutation in PRPF31 is hypostatic to a trait acting on CNOT3, with the RP11 phenotype only being observed when there is homozygous (recessive) inheritance of the higher expressivity CNOT3 ("symptomatic" or risk) allele. PMID: 24116917
    16. identification of the novel "A" insertion mutation in exon9 at chr19:54629961-54629961 in PRPF31 can provide further genetic evidence for clinical test for adRP and JMD. PMID: 24244300
    17. Two novel PRPF31 mutations including a complex insertion-deletion mutation have been identified in Chinese families with retinitis pigmentosa. PMID: 24319336
    18. We have identified a novel 14-bp deletion in PRPF31 as the genetic driver of adRP in a large Mexican family that exhibits nonpenetrance and variable expressivity, known properties of this locus. PMID: 24595387
    19. The molecular and clinical features of a family with a novel 3-base insertion, c.914_915insTGT (p.Val305_Asp306insVal) in exon 9 of PRPF31 are described to illustrate the salient clinical features of mutations in this gene. PMID: 23343310
    20. In asymptomatic carriers CNOT3 is expressed at low levels, allowing higher amounts of wild-type PRPF31 transcripts to be produced and preventing manifestation of retinal degeneration. PMID: 23144630
    21. Novel PRPF31 mutations associated with Chinese autosomal dominant retinitis pigmentosa patients occur at a relatively high frequency. PMID: 23288994
    22. The present study describes mapping of a locus for non-syndromic adRP at 19q13.42 (RP11 locus) in a family of Indian origin and identifies a novel deletion, c.59_65del7, in PRPF31 within the mapped interval. PMID: 23041261
    23. Characterization of the core promoters controlling the expression of PRPF31 and TFPT, a bi-directional gene-pair. PMID: 22723017
    24. This study describes two large deletions, one in a previously reported family and one in a new family: the latter represents the largest deletion yet described on chromosome 19 and the first report of the involvement of VSTM-1. PMID: 21715351
    25. RP-PRPF defects affect the stoichiometry of spliceosomal small nuclear RNAs. In cells with PRPF31 mutations there was no lymphoblasts with PRPF31 mutations correctly assembled tri-snRNPs, but in a less efficient manner compared with controls. PMID: 21378395
    26. CTNNBL1 is a novel nuclear localization sequence-binding protein that recognizes RNA-splicing factors CDC5L and Prp31 PMID: 21385873
    27. extended the mutation spectrum of PRPF31 and as previously reported in other populations, it is a major cause of autosomal dominant rod-cone dystrophy in France. PMID: 20939871
    28. Protein 61K, encoded by a gene (PRPF31) linked to autosomal dominant retinitis pigmentosa, is required for U4/U6*U5 tri-snRNP formation and pre-mRNA splicing. PMID: 11867543
    29. Two missense mutations in PRPF31 (A194E and A216P) linked to autosomal dominant retinitis pigmentosa substantially hinder translocation of PRPF31 into the nucleus. PMID: 12444105
    30. Deletion in the pre-mRNA splicing gene PRPF31 causes autosomal dominant retinitis pigmentosa PMID: 12923864
    31. Partial penetrance in RP11 could be due to the coinheritance of a PRPF31 gene defect and a low-expressed wild-type allele PMID: 14507862
    32. A novel splicing mutation (IVS5-1G>A) in the pre-mRNA splicing-factor gene PRPF31 causes retinitis pigmentosa in a large Chinese family. The mutation results in a truncated protein of PRPF31. PMID: 15162096
    33. in the absence of either hPrp31 or hPrp6, U4/U6 di-snRNPs as well as p110 accumulate in Cajal bodies PMID: 15257298
    34. Our results demonstrate that mutations in PRPF31 gene affect rhodopsin (RHO) pre-mRNA splicing and reveal a link between PRPF31 and RHO, two major genes in autosomal dominant retinitis pigmentosa. PMID: 15659613
    35. A novel splice site mutation in the PRPF31 gene caused retinitis pigmentosa (RP) in the four-generation Chinese RP family. PMID: 15924690
    36. The 1142delG and 1155-1159delGGACG/insAGGGATT mutations in the PRPF31 gene cause RP. PMID: 16139010
    37. Data demonstrate that His-tagged PRPF31 interacts with importin beta1 for translocation to the nucleus, with no requirement for importin alpha1. PMID: 16427773
    38. This mutation provides evidence that haploinsufficiency rather than aberrant function of mutated proteins is cause of disease in these adRP patients with mutations in PRPF31 gene. PMID: 16636657
    39. We describe a novel PRPF31 mutation and present the first case of a homozygous mutation in the RPGR gene in a female individual. PMID: 16917484
    40. MLPA (multiplex ligation-dependent probe amplification) was used to identify genomic rearrangements in PRPF31 in five families, suggesting a frequency of approximately 2.5%. PMID: 17003455
    41. Although the frequency of mutations in the PRPF31 gene is about 2.5% in Japanese families with adRP, it is possible that c.1142delG is a common mutation among Japanese patients with adRP associated with mutations in the PRPF31 gene. PMID: 17295140
    42. Mutations in PRPF31 causing adRP (autosomal dominant retinitis pigmentosa) were present in nearly 5% of a mixed U.K. population. PMID: 17325180
    43. structural and biochemical analyses of hPrp31-15.5K-U4 snRNA complexes show how the conserved Nop domain in hPrp31 maintains high RNP binding selectivity despite relaxed RNA sequence requirements PMID: 17412961
    44. a new mutation in the PRPF31 gene is described, together with the clinical phenotype of dominant retinitis pigmentosa PMID: 17895420
    45. Our studies identify a novel splicing mutation in PRPF31 associated with adRP and suggest that the penetrance of RP11 mutations may be correlated with the expression level of the PRPF31 mRNA. PMID: 18177735
    46. pathogenic effect of PRPF31 mutations is likely due to haploinsufficiency rather than to gain of function PMID: 18317597
    47. The RP11 missense mutations exert their pathology mainly via a mechanism based on protein insufficiency due to protein insolubility, but there is also a minor direct negative effect on function. PMID: 18431455
    48. RHO, PRPF31, RP1, and IMPDH1 were screened and causative mutations were identifiedin 4% of isolated and 2% of autosomal dominant forms of retinitis pigmentosa patients from India. PMID: 18552984
    49. PRPF31 mRNA expression and consequently the penetrance of PRPF31 mutations is managed by diffusible compounds encoded by at least two modifiers, acting in a co-regulatory system on both PRPF31 alleles during transcription. PMID: 18640990
    50. Ala216Pro mutation destabilizes the hPrp31 protein structure and reduces its interaction with snRNP binding. PMID: 19293337

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  • 相關疾?。?/div>
    Retinitis pigmentosa 11 (RP11)
  • 亞細胞定位:
    Nucleus. Nucleus speckle. Nucleus, Cajal body.
  • 蛋白家族:
    PRP31 family
  • 組織特異性:
    Ubiquitously expressed.
  • 數據庫鏈接:

    HGNC: 15446

    OMIM: 600138

    KEGG: hsa:26121

    STRING: 9606.ENSP00000324122

    UniGene: Hs.515598



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