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

ADSL Antibody

  • 中文名稱:
    ADSL兔多克隆抗體
  • 貨號:
    CSB-PA001397ESR2HU
  • 規格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: ADSL antibody at 2µg/ml
      Lane 1: Hela whole cell lysate
      Lane 2: Mouse heart tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 55, 49 kDa
      Observed band size: 55 kDa
    • Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA001397ESR2HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ADSL Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    ADSL
  • 別名:
    Adenylosuccinase antibody; Adenylosuccinate lyase antibody; ADSL antibody; AMPS antibody; ASase antibody; ASL antibody; OTTHUMP00000199172 antibody; OTTHUMP00000199173 antibody; PUR8_HUMAN antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Adenylosuccinate lyase protein (1-310AA)
  • 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, WB, IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
    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 two non-sequential steps in de novo AMP synthesis: converts (S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate (SAICAR) to fumarate plus 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide, and thereby also contributes to de novo IMP synthesis, and converts succinyladenosine monophosphate (SAMP) to AMP and fumarate.
  • 基因功能參考文獻:
    1. Case Report: Malaysian patient compound heterozygous for two novel ADSL mutations giving rise to adenylosuccinate lyase deficiency. PMID: 20177786
    2. Missense mutations in the adenylosuccinate lyase is associated with Adenylosuccinate lyase deficiency, an inborn error of purine metabolism characterized by neurological and physiological symptoms. PMID: 23714113
    3. structural and biochemical characterization data of WT and mutant R303C ADSL by enzyme kinetics, product binding by isothermal titration calorimetry and X-ray crystallography to reveal the effects of the R303C mutation that results in a nonparallel reduction in enzyme activity PMID: 22812634
    4. Results proved in cultured skin fibroblasts from patients with AICA-ribosiduria and ADSL deficiency that various mutations of ADSL destabilize to various degrees purinosome assembly and found that the ability to form purinosomes correlates with clinical phenotypes of individual ADSL patients. PMID: 22180458
    5. D-ribose administration in Polish patients with adenylosuccinate lyase deficiency was accompanied by neither reduction in seizure frequency nor growth enhancement. PMID: 18649008
    6. the cases of the only three children diagnosed to date in the United Kingdom with adenylosuccinate lyase deficiency PMID: 20933180
    7. Mutation of a nuclear respiratory factor 2 binding site in the 5' untranslated region of the ADSL gene in three patients with adenylosuccinate lyase deficiency. PMID: 12016589
    8. Mutations at position 276 result in structurally impaired adenylosuccinate lyases which are assembled into the defective tetramers associated with the mild variant of ADSL deficiency in humans. PMID: 12590570
    9. Variable expression of ADSL deficiency is reported in three patients belonging to a family which originates from Portugal. PMID: 12833398
    10. a mutation in adenylosuccinate lyase may be associated with autism PMID: 15471876
    11. case report of adenylosuccinate lyase deficiency shows a mutation in ASDL PMID: 15571235
    12. cloning, expression and purification of catalytically active human adenylosuccinate lyase PMID: 16973378
    13. ADSL deficiency may present with prenatal growth restriction, fetal and neonatal hypokinesia, and rapidly fatal neonatal encephalopathy. PMID: 17188615
    14. Analysis of the ADSL gene showed a R426H mutation in four unrelated patients with metabolic diseases. PMID: 18524658
    15. Biochemical and biophysical analysis of five disease-associated human adenylosuccinate lyase mutants. PMID: 19405474

    顯示更多

    收起更多

  • 相關疾病:
    Adenylosuccinase deficiency (ADSLD)
  • 蛋白家族:
    Lyase 1 family, Adenylosuccinate lyase subfamily
  • 組織特異性:
    Ubiquitously expressed. Both isoforms are produced by all tissues. Isoform 2 is 10-fold less abundant than isoform 1.
  • 數據庫鏈接:

    HGNC: 291

    OMIM: 103050

    KEGG: hsa:158

    STRING: 9606.ENSP00000216194

    UniGene: Hs.75527



主站蜘蛛池模板: 国产又粗又猛又爽的视频a片| 国产av无码专区国产乱码| 亚洲 中文 欧美 日韩 在线| 超清无码av最大网站| 国产精品无码永久免费888| 免费人成小说在线观看网站| 乱子伦一区二区三区| 国精品人妻无码一区二区三区d3| 国色天香国产精品| 国产精品久久久久久影视不卡| 亚洲色婷婷一区二区三区| 久久九九久精品国产日韩经典 | 精品久久久久久久中文字幕| 中文字幕日韩欧美一区二区三区| 99精品国产兔费观看久久| 亚洲超清无码制服丝袜无广告| 日本大香伊一区二区三区| 亚洲最大天堂无码精品区| 国产成人综合久久精品推下载| 色婷婷狠狠97成为人免费| 奶头好大狂揉60分钟视频| 国产麻豆精品一区二区三区v视界| 妖精色av无码国产在线看| 国产多人群p刺激交换视频| 一二三四在线视频观看社区| 国产精品a成v人在线播放| 国产 校园 另类 小说区| 久久不见久久见免费视频观看| 又粗又大内射免费视频小说| 国产精品制服| 亚洲国产精品成人影片久久| av无码av天天av天天爽| 少妇熟女视频一区二区三区| 国产丰满乱子伦无码专区| av无码久久久久不卡网站蜜桃| 日日碰狠狠躁久久躁蜜桃| 国产av国内精品jk制服| 狂野欧美激情性xxxx按摩| 欧美人妻aⅴ中文字幕| 又色又爽又高潮免费观看| 好男人在在线社区www在线影院|