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TSEN54 Antibody

  • 货号：
    CSB-PA272793
  • 规格：
    ￥2024
  • 图片：
    • 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：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TSEN54 Polyclonal antibody
  • Uniprot No.：
    Q7Z6J9
  • 基因名：
    TSEN54
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from internal of Human TSEN54.
  • 免疫原种属：
    Homo sapiens (Human)
  • 克隆类型：
    Polyclonal
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:3000
    IHC 1:50-1:100
  • 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：
    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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Pontocerebellar hypoplasia 4 (PCH4); Pontocerebellar hypoplasia 2A (PCH2A); Pontocerebellar hypoplasia 5 (PCH5)
  • 亚细胞定位：
    Nucleus. Nucleus, nucleolus.
  • 蛋白家族：
    SEN54 family
  • 数据库链接：

    HGNC: 27561

    OMIM: 225753

    KEGG: hsa:283989

    STRING: 9606.ENSP00000327487

    UniGene: Hs.378501