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  4. TSEN54 Antibody

TSEN54 Antibody

  • 中文名稱：
    TSEN54兔多克隆抗體
  • 貨號：
    CSB-PA272793
  • 規(guī)格：
    ￥2024
  • 圖片：
    • Western blot analysis of extracts from 3T3 cells and Jurkat cells, using TSEN54 antibody.
    • Immunohistochemistry analysis of paraffin-embedded human lung carcinoma tissue, using TSEN54 antibody.
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) TSEN54 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    TSEN54
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Synthesized peptide derived from internal of Human TSEN54.
  • 免疫原種屬：
    Homo sapiens (Human)
  • 克隆類型：
    Polyclonal
  • 純化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA,WB,IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:3000
    IHC 1:50-1:100
  • Protocols：
  • 儲(chǔ)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(chǎn)品評價(jià)

靶點(diǎn)詳情

  • 功能：
    Non-catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5' and 3' splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2',3' cyclic phosphate and 5'-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural features of the tRNA body. The tRNA splicing endonuclease is also involved in mRNA processing via its association with pre-mRNA 3'-end processing factors, establishing a link between pre-tRNA splicing and pre-mRNA 3'-end formation, suggesting that the endonuclease subunits function in multiple RNA-processing events.
  • 基因功能參考文獻(xiàn)：
    1. TSEN54 gene-related pontocerebellar hypoplasia type 2 presented with exaggerated startle response in cousins. PMID: 26701950
    2. A novel heterozygous mutation was found in the TSEN54 gene by c.254A > T(+) (p.E85V), which may be a new subtype of hereditary ataxia PMID: 24938831
    3. TSEN54 mutation causes a severe form of pontocerebellar hypoplasia type 1 in a family. PMID: 21468723
    4. The results demonistrated that not all cases of clinically defined pontocerebellar hypoplasia-4 result from mutations in TSEN54. PMID: 21383226
    5. We confirm that the common p.A307S mutation in TSEN54 is responsible for most of the patients with a PCH2 phenotype. PMID: 20956791
    6. In two subtypes, PCH2 and PCH4, we identified mutations in three of the four different subunits of the tRNA-splicing endonuclease complex. PMID: 18711368

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  • 相關(guān)疾?。?/div>
    Pontocerebellar hypoplasia 4 (PCH4); Pontocerebellar hypoplasia 2A (PCH2A); Pontocerebellar hypoplasia 5 (PCH5)
  • 亞細(xì)胞定位：
    Nucleus. Nucleus, nucleolus.
  • 蛋白家族：
    SEN54 family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 27561

    OMIM: 225753

    KEGG: hsa:283989

    STRING: 9606.ENSP00000327487

    UniGene: Hs.378501