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AARS2 Antibody

  • 中文名稱：
    AARS2兔多克隆抗體
  • 貨號(hào)：
    CSB-PA711453ESR2HU
  • 規(guī)格：
    ￥440
  • 促銷：
    小規(guī)格抗體限時(shí)一口價(jià)
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA711453ESR2HU at dilution of 1:100
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) AARS2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    AARS2
  • 別名：
    AARS2 antibody; AARSL antibody; KIAA1270 antibody; Alanine--tRNA ligase antibody; mitochondrial antibody; EC 6.1.1.7 antibody; Alanyl-tRNA synthetase antibody; AlaRS antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Alanine--tRNA ligase, mitochondrial protein (841-985AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 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

  • 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.
  • 基因功能參考文獻(xiàn)：
    1. Two AARS2 variants, (c.2872C > T) and (c.1774C > T), were identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
    2. Three patients with ovarioleukodystrophy, carrying AARS2 compound heterozygous mutations have been found. PMID: 29749055
    3. Missense variants in the AARS2 gene are the likely cause of the retinopathy and optic atrophy in this patient. This finding expands the phenotypic spectrum of the AARS2 gene. PMID: 28820624
    4. Mutations in AARS2 in a Series of CSF1R-Negative Patients With Adult-Onset Leukoencephalopathy With Axonal Spheroids and Pigmented Glia. PMID: 27749956
    5. This paper documented a new, nonsense AARS2 gene mutation (c.578T>G, p.Leu193*) and a known missense mutation (c.595C>T, p.Arg199Cys) associated with leukoencephalopathy in a male patient. PMID: 27734837
    6. we describe a Japanese woman with novel compound heterozygous mutations in AARS2, the first report of leukodystrophy caused by AARS2 mutations in Asia. PMID: 27251004
    7. A new phenotype caused by AARS2 mutations is characterized by leukoencephalopathy and ovarian failure in female patients, indicating that the phenotypic spectrum associated with AARS2 variants is much wider than previously reported. PMID: 24808023
    8. Mutations in AARS2 found in lethal mitochondrial myopathy PMID: 22277967

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  • 相關(guān)疾病：
    Combined oxidative phosphorylation deficiency 8 (COXPD8); Leukoencephalopathy, progressive, with ovarian failure (LKENP)
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 21022

    OMIM: 612035

    KEGG: hsa:57505

    STRING: 9606.ENSP00000244571

    UniGene: Hs.158381