在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

AARS2 Antibody

  • 中文名稱:
    AARS2兔多克隆抗體
  • 貨號:
    CSB-PA711453ESR2HU
  • 規格:
    ¥440
  • 圖片:
    • Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA711453ESR2HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) AARS2 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 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
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Alanine--tRNA ligase, mitochondrial protein (841-985AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    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.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols:
  • 儲存條件:
    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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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:
    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

    顯示更多

    收起更多

  • 相關疾病:
    Combined oxidative phosphorylation deficiency 8 (COXPD8); Leukoencephalopathy, progressive, with ovarian failure (LKENP)
  • 亞細胞定位:
    Mitochondrion.
  • 蛋白家族:
    Class-II aminoacyl-tRNA synthetase family
  • 數據庫鏈接:

    HGNC: 21022

    OMIM: 612035

    KEGG: hsa:57505

    STRING: 9606.ENSP00000244571

    UniGene: Hs.158381



主站蜘蛛池模板: 日韩一本之道一区中文字幕| 日本极品少妇videossexhd| 欧美人妻日韩精品| 国模大尺度啪啪| 日本韩国的免费观看视频| 亚洲欧美日韩国产精品一区| 天天爽夜夜爱| 亚洲精品一区中文字幕乱码| 久久综合少妇11p| 免费无码av片流白浆在线观看| 男人j进入女人j内部免费网站| 亚洲午夜精品一区二区| 色欲av亚洲情无码av蜜桃| 国产老熟女狂叫对白| 精品无码黑人又粗又大又长| 成人午夜国产内射主播| 9999国产精品欧美久久久久久| 丝袜美腿亚洲一区二区| 99久久99久久精品国产片果冻| av大片在线无码永久免费| 亚洲性人人天天夜夜摸18禁止| 性欧美丰满熟妇xxxx性| 白又丰满大屁股bbbbb| 久久精品熟女亚州av麻豆| 亚洲国产成人在人网站天堂| 无码视频一区二区三区在线观看 | 国产精品特黄aaaa片在线观看 | 成人乱人乱一区二区三区| 亚洲色老汉av无码专区最| 国产精品人妻一区二区高| 亚洲精品乱码久久久久久蜜桃图片| 亚洲 日韩 国产 中文有码| 成年动漫18禁无码3d动漫 | 亚洲午夜福利在线观看| 女人喷水高潮时的视频网站| 人人爽人人爽人人片a免费| 精品丰满人妻无套内射| 大肉大捧一进一出好爽app| 国产成人美女视频网站| 4444亚洲人成无码网在线观看 | 美女裸体自慰在线观看|