PRPF3 Antibody, FITC conjugated

說明書

中文名稱：

PRPF3兔多克隆抗體, FITC偶聯
貨號：

CSB-PA018763LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) PRPF3 Polyclonal antibody
Uniprot No.：

O43395
基因名：

PRPF3
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human U4/U6 small nuclear ribonucleoprotein Prp3 protein (1-200AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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).
基因功能參考文獻：
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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相關疾?。?/div>
Retinitis pigmentosa 18 (RP18)
亞細胞定位：

Nucleus. Nucleus speckle.
組織特異性：

Highly expressed in retina, liver, kidney and blood. Detected at lower levels in heart and brain.
數據庫鏈接：

HGNC: 17348

OMIM: 601414

KEGG: hsa:9129

STRING: 9606.ENSP00000315379

UniGene: Hs.11776

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