PRPF3 Antibody, HRP conjugated
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中文名稱：PRPF3兔多克隆抗體, HRP偶聯
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貨號：CSB-PA018763LB01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) PRPF3 Polyclonal antibody
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Uniprot No.：
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基因名：PRPF3
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別名：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
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human U4/U6 small nuclear ribonucleoprotein Prp3 protein (1-200AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：HRP
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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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).
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基因功能參考文獻：
- 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
- 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
- RP-PRPF defects affect the stoichiometry of spliceosomal small nuclear RNAs. Mutant PRPF3 proteins stably associated with tri-snRNPs. PMID: 21378395
- A mutation in the PRPF3 gene is rare compared to other genes causing autosomal dominant retinitis pigmentosa. PMID: 20309403
- Mutations in HPRP3, a third member of pre-mRNA splicing factor genes, implicated in autosomal dominant retinitis pigmentosa. PMID: 11773002
- role in the recruitment of Hprp4p for the U4/U6 snRNP assembly PMID: 11971898
- 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
- 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
- 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
- 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
- splicing factor PRPF3 mutations cause retinal degeneration and form detrimental aggregates in photoreceptor cells PMID: 17517693
- 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
- splicing activity is significantly influenced by the CK2-hPrp3p interaction PMID: 18026141
- TASP1, EPS15R, and PRPF3 expression were significantly induced in HCCs of transgenic EGF2B mice as was P2 promoter-driven HNF4alpha PMID: 18395097
- These data support the notion about individual roles for CK2alpha and CK2alpha' in the splicing process. PMID: 18553058
- The human HPRP3 gene, the orthologue of the yeast pre-mRNA splicing factor (PRP3) PMID: 11773002
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相關疾病：Retinitis pigmentosa 18 (RP18)
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亞細胞定位：Nucleus. Nucleus speckle.
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組織特異性：Highly expressed in retina, liver, kidney and blood. Detected at lower levels in heart and brain.
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數據庫鏈接：
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