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DARS2 Antibody

  • 货号：
    CSB-PA747490LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: DARS2 antibody at 10μg/ml + MCF-7 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 74 kDa
      Observed band size: 74 kDa
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA747490LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) DARS2 Polyclonal antibody
  • Uniprot No.：
    Q6PI48
  • 基因名：
    DARS2
  • 别名：
    DARS2 antibody; Aspartate--tRNA ligase antibody; mitochondrial antibody; EC 6.1.1.12 antibody; Aspartyl-tRNA synthetase antibody; AspRS antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Aspartate--tRNA ligase, mitochondrial protein (489-628AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，DARS2 Antibody (CSB-PA747490LA01HU)，的标记方式是Non-conjugated。对于DARS2 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA747490LB01HU DARS2 Antibody, HRP conjugated ELISA
    FITC CSB-PA747490LC01HU DARS2 Antibody, FITC conjugated
    Biotin CSB-PA747490LD01HU DARS2 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • 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

  • 基因功能参考文献：
    1. Whole exome sequencing of the proband identified two compound heterozygous variants (NM_018122.4:c.1762C > G and c.563G > A) in DARS2 PMID: 30352563
    2. Results show that DARS2 is strongly upregulated in hepatocellular carcinoma (HCC) and is associated with HCC progression. DARS2 promotes HCC tumorigenesis by accelerating cell cycle progression and attenuating cell apoptosis. PMID: 29052520
    3. Mutations with mild effects on solubility occur in patients as allelic combinations whereas those with strong effects on solubility or on aminoacylation are necessarily associated with a partially functional allele. PMID: 26620921
    4. This study identified DARS2-associated leukoencephalopathy with hypomyelination with brainstem and spinal cord involvement and leg spasticity. PMID: 25527264
    5. 60 different DARS2 mutations were identified in 78 patients with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, 13 of which have not been reported before PMID: 24566671
    6. Cognitive impairment seems to be common among patients with leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate and DARS2 mutations PMID: 23652419
    7. A comparison of biophysical properties of human mitochondrial aspartyl-tRNA synthetase, HsaDRS2, with them to those of a bacterial (E. coli) homolog, EcoDRS. PMID: 23275545
    8. Pathogenic mutations causing LBSL affect mitochondrial aspartyl-tRNA synthetase in diverse way PMID: 23216004
    9. We describe two new cases of Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate with a novel pathogenic mutation in the DARS2 gene PMID: 21792730
    10. Case demonstrates that DARS2 mutation homozygosity is not lethal, as suggested earlier, but compatible with a rather benign disease course. PMID: 21749991
    11. Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation is associated with cell-type-dependent splicing of mtAspRS mRNA. PMID: 22023289
    12. A novel homozygous mutation of DARS2 may cause a severe LBSL (Leukoencephalopathy with brain stem and spinal cord involvement with lactate elevation) variant. PMID: 21815884
    13. This report describes two novel heterozygote composite mutations in the DARS2 gene PMID: 20878420
    14. DARS2 mutations cause childhood-to-adolescence onset leucoencephalopathy, but they do not seem to be associated with multiple sclerosis. PMID: 19592391
    15. The gene for mitochondrial aspartyl-tRNA synthetase is described and the initial characterization of the enzyme is reported. Genes for the remaining missing human synthetases have also been found with the exception of glutaminyl-tRNA synthetase. PMID: 15779907
    16. Mutations in DARS2, which encodes mitochondrial aspartyl-tRNA synthetase, in affected individuals with leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation. PMID: 17384640

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  • 相关疾病：
    Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family, Type 1 subfamily
  • 数据库链接：

    HGNC: 25538

    OMIM: 610956

    KEGG: hsa:55157

    STRING: 9606.ENSP00000355086

    UniGene: Hs.647707