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HARS Antibody

  • 货号：
    CSB-PA010137GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P12081
  • 基因名：
    HARS
  • 别名：
    cytoplasmic antibody; EC 6.1.1.21 antibody; FLJ20491 antibody; HARS antibody; HisRS antibody; Histidine tRNA ligase, cytoplasmic antibody; histidine translase antibody; Histidine tRNA ligase antibody; Histidine--tRNA ligase antibody; Histidyl tRNA synthetase antibody; Histidyl-tRNA synthetase antibody; HRS antibody; Human histidyl tRNA synthetase homolog (HO3) mRNA complete cds antibody; SYHC_HUMAN antibody; USH3B antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human HARS
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IF
  • 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：
    Catalyzes the ATP-dependent ligation of histidine to the 3'-end of its cognate tRNA, via the formation of an aminoacyl-adenylate intermediate (His-AMP). Plays a role in axon guidance.
  • 基因功能参考文献：
    1. This study suggests that the human HisRS genes, while descending from a common ancestor with dual function for both types of tRNA(His), have acquired highly specialized tRNA recognition properties through evolution. PMID: 28321488
    2. Despite the similar kinetics, differential scanning fluorimetry revealed that Y454S is less thermally stable than Wild Type HARS, and cells from Y454S patients grown at elevated temperatures demonstrate diminished levels of protein synthesis compared to those of Wild Type cells. The thermal sensitivity associated with the Y454S mutation represents a biochemical basis for understanding Usher Syndrome Type IIIB. PMID: 28632987
    3. Loss of function mutations in histidyl-tRNA synthetase cause a spectrum of inherited peripheral neuropathies PMID: 26072516
    4. Data suggest that by comparing human and trypanosomatid histidyl-tRNA synthetases (HisRS) may provide opportunities for developing specific inhibitors of Trypanosoma brucei HisRS. PMID: 25151410
    5. Secreted histidyl-tRNA synthetase splice variants elaborate major epitopes for autoantibodies in inflammatory myositis. PMID: 24898250
    6. Data indicate that higher anti-Jo1 levels were associated with disease severity in antisynthetase syndrome (ASS) patients. PMID: 24286268
    7. Non-Jo-1 anti-synAb positive patients have decreased survival compared with Jo-1 patients. PMID: 23422076
    8. Findings suggest that histidyl-tRNA synthetase (HARS) is associated with axonal peripheral neuropathy. PMID: 22930593
    9. The crystal structure of a novel splice variant of a tRNA synthetase lacking the entire catalytic domain. PMID: 22958643
    10. Although anti-Jo1 positive patients with antisynthetase syndrome share features of patients with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. PMID: 22326685
    11. Study identified sequence variants in the known disease-causing genes SLC6A3 and FLVCR1, and present evidence to strongly support the pathogenicity of variants identified in TUBGCP6, BRAT1, SNIP1, CRADD, and HARS. PMID: 22279524
    12. genomic organization of the HARS locus and mapping of transcripts originating from a bi-directional promoter controlling the differential expression of these gene PMID: 12056811
    13. Demonstrating histidyl-tRNA synthetase (Jo-1)-specific T cell responses represents a key step in establishing the hypothesis that Jo-1 drives T cell-mediated autoimmunity in Jo-1+ polymyositis. PMID: 12471150
    14. the TSG101 interaction with HRS is a crucial step in endocytic down-regulation of mitogenic signaling and this interaction may have a role in linking the functions of early and late endosomes PMID: 12802020
    15. A proteolytically sensitive conformation of HisRS exists in the lung, the target tissue associated with this autoantibody response. PMID: 17665459
    16. clinical and prognostic profiles of 45 patients displaying anti-histidyl-tRNA synthetase autoantibodies were determined PMID: 17785330

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  • 相关疾病：
    Usher syndrome 3B (USH3B); Charcot-Marie-Tooth disease 2W (CMT2W)
  • 亚细胞定位：
    Cytoplasm.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family
  • 组织特异性：
    Brain, heart, liver and kidney.
  • 数据库链接：

    HGNC: 4816

    OMIM: 142810

    KEGG: hsa:3035

    STRING: 9606.ENSP00000425634

    UniGene: Hs.528050