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AARS Antibody

  • 货号：
    CSB-PA208319
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using CSB-PA208319(AARS Antibody) at dilution 1/60, on the right is treated with synthetic peptide. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    P49588
  • 基因名：
    AARS
  • 别名：
    AARS antibody; AI316495 antibody; Alanine tRNA ligase 1, cytoplasmic antibody; Alanine tRNA ligase antibody; Alanine tRNA ligase cytoplasmic antibody; Alanine--tRNA ligase antibody; Alanyl tRNA synthetase antibody; Alanyl tRNA synthetase cytoplasmic antibody; Alanyl-tRNA synthetase antibody; AlaRS antibody; C76919 antibody; CMT2N antibody; cytoplasmic antibody; EC 6.1.1.7 antibody; MGC37368 antibody; Renal carcinoma antigen NY REN 42 antibody; Renal carcinoma antigen NY-REN-42 antibody; SYAC_HUMAN antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Rat
  • 免疫原：
    Synthetic peptide of Human AARS
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:50-1:200
  • 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

  • 功能：
    Catalyzes the attachment of alanine to tRNA(Ala) in a two-step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain.
  • 基因功能参考文献：
    1. A large sequence divergence of the C-terminal domain (C-Ala) reshaped C-Ala in a way that changed the global architecture of alanyl-tRNA synthetase (AlaRS). This reshaping removed the role of C-Ala in prokaryotes for docking tRNA and instead repurposed it to form a dimer interface presenting a DNA-binding groove. PMID: 27911835
    2. Number of missense mutations in AARS expand the clinical spectrum and provide pheno-genotypic correlations in AARS-related neuropathies. PMID: 26032230
    3. A novel mutation in alanyl-tRNA synthetase causes a mild myeloneuropathy, a novel phenotype for patients with mutations in one of the tRNA synthetase genes. PMID: 25904691
    4. Loss-of-function alanyl-tRNA synthetase mutations cause an autosomal-recessive early-onset epileptic encephalopathy with persistent myelination defect. PMID: 25817015
    5. the pathological consequences of diminished tRNA synthetase editing activity, and thus translational infidelity, are dependent on the cell type and the extent of editing disruption PMID: 25422440
    6. in a family with distal hereditary motor neuropathy (dHMN), all 4 affected family members had a heterozygous missense mutation c.2677G>A (p.D893N) of (AARS), not found in the 4 unaffected members and control subjects; conclude AARS mutation caused dHMN in a Chinese family; AARS mutations result in not only a CMT phenotype but also a dHMN phenotype PMID: 22573628
    7. Methylation-mediated deamination of a CpG dinucleotide gives rise to the recurrent p.Arg329His alanyl-tRNA synthetase mutation in patients with Charcot-Marie-Tooth disease type 2N (CMT2N). PMID: 22009580
    8. We show here that mutations in AARS2 cause perinatal or infantile cardiomyopathy with near-total combined mitochondrial respiratory chain deficiency in the heart. PMID: 21549344
    9. cytoplasmic Alanyl-tRNA synthetase may have a role in dominant axonal Charcot-Marie-Tooth disease, as shown by its mutation in a major determinant for binding and aminoacylation PMID: 20045102

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  • 相关疾病：
    Charcot-Marie-Tooth disease 2N (CMT2N); Epileptic encephalopathy, early infantile, 29 (EIEE29)
  • 亚细胞定位：
    Cytoplasm.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family
  • 数据库链接：

    HGNC: 20

    OMIM: 601065

    KEGG: hsa:16

    STRING: 9606.ENSP00000261772

    UniGene: Hs.315137