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PARS2 Antibody, FITC conjugated

  • 中文名稱：
    PARS2兔多克隆抗體, FITC偶聯(lián)
  • 貨號(hào)：
    CSB-PA745334LC01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) PARS2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    PARS2
  • 別名：
    PARS2 antibody; Probable proline--tRNA ligase antibody; mitochondrial antibody; EC 6.1.1.15 antibody; Prolyl-tRNA synthetase antibody; ProRS antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Probable proline--tRNA ligase, mitochondrial protein (126-258AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 基因功能參考文獻(xiàn)：
    1. study provides further evidence for validating the role of PARS2 in the pathology of related infantile-onset encephalopathy, contributing to the phenotypic features of this condition, and providing clinical and molecular insight for the diagnosis of this disease entity. PMID: 29915213
    2. Depending on available data of six patients from three families, we describe a phenotype linked to PARS2 pathological variants. This phenotype includes: early epileptic encephalopathy, infantile spasms, and Alpers-like brain MRI changes with predominance of frontal cerebral volume loss (FCVL-AS). PMID: 29410512
    3. The individuals with PARS2 and NARS2 mutations demonstrate similar neurological features as those previously reported, with diversity in clinical presentation such as hearing loss and seizure type. PMID: 28077841
    4. The PARS2 gene produced a significant association signal in a recent replication study of all known CRS-associations and is now the first human gene to be re-sequenced within CRS research. PARS2 showed an accumulation of low-frequency variants in CRS patients compared to background populations. PMID: 27348859
    5. unusual 2.0 A structure showing that ATP directly locks onto and orients two parts of halofuginone (HF) onto human ProRS, so that one part of HF mimics bound proline and the other mimics the 3' end of bound tRNA. PMID: 23263184

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  • 亞細(xì)胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 30563

    OMIM: 612036

    KEGG: hsa:25973

    STRING: 9606.ENSP00000360327

    UniGene: Hs.380169