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

ECHS1 Antibody

  • 中文名稱:
    ECHS1兔多克隆抗體
  • 貨號:
    CSB-PA007382ESR1HU
  • 規格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: ECHS1 antibody at 3.03µg/ml
      Lane 1: Mouse liver tissue
      Lane 2: Mouse skeletal muscle tissue
      Lane 3: Hela whole cell lysate
      Lane 4: HepG2 whole cell lysate
      Lane 5: MCF-7 whole cell lysate
      Lane 6: Jurkat whole cell lysate
      Lane 7: A549 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 32 kDa
      Observed band size: 32 kDa
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA007382ESR1HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ECHS1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    ECHS1
  • 別名:
    ECHS1Enoyl-CoA hydratase antibody; mitochondrial antibody; EC 4.2.1.17 antibody; Enoyl-CoA hydratase 1 antibody; Short-chain enoyl-CoA hydratase antibody; SCEH antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Enoyl-CoA hydratase, mitochondrial protein (28-290AA)
  • 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 1:1000-1:5000
    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:
    Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate.
  • 基因功能參考文獻:
    1. Due to enoyl coenzyme A hydratase, short chain, 1, mitochondria (ECHS1) deficiency. PMID: 28409271
    2. exceeding nutrients suppress Enoyl-CoA hydratase-1 (ECHS1) activity by inducing its acetylation resulting in accumulation of fatty acids and branched-chain amino acids and oncogenic mTOR activation PMID: 28878358
    3. ECHS1 mutations phenotype might be milder than reported earlier, compatible with prolonged survival, and also includes isolated paroxysmal exercise-induced dystonia. PMID: 27090768
    4. Whole exome sequencing identified heterozygous ECHS1 mutations c.836T>C (novel) and c.8C>A for short-chain enoyl-CoA hydratase (SCEH) deficiency of which 1/2 of the cases are associated with secondary lymphocyte pyruvate dehydrogenase complex deficiency.[review] PMID: 28202214
    5. This is the first report of homozygosity for a truncating mutation in ECHS1, which may explain the severe phenotype. Our report highlights the need to consider SCEH deficiency in patients with lethal neonatal lactic acidosis PMID: 27905109
    6. Human ECHS1 catalyses the hydration of five substrates via different metabolic pathways, with the highest specificity for crotonyl-CoA and the lowest specificity for tiglyl-CoA PMID: 26251176
    7. These results suggested that ECHS1 may promote cell proliferation in hepatocellular carcinoma in an EGFR-dependent manner. PMID: 25760819
    8. In conclusion, the results of the present study suggested that ECHS1 may have an important role in colorectal cancer cell proliferation and migration PMID: 25739098
    9. identification of four additional patients with mutations in ECHS1 highlights the importance of the valine degradation pathway in Leigh syndrome PMID: 26099313
    10. ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome. PMID: 25393721
    11. ECHS1 may play important roles in gastric cancer cell proliferation and migration through PKB- and GSK3b-related signaling pathways. PMID: 25338767
    12. The study demonstrated that ECHS1 mutations result in ECHS1 deficiency and are another cause of Leigh disease in two siblings. PMID: 25125611
    13. ECHS1 knockdown reduced cell viability and enhanced cisplatin-induced apoptosis in hepatocellular carcinoma cells. PMID: 23879543
    14. The results confirmed that small hepatitis B surface antigen (SHBs) interacted with ECHS1. PMID: 23275097
    15. The coexistence of HBs and ECHS1 enhances HepG2 cell apoptosis, affects ECHS1 localization in the mitochondria and induces apoptosis by decreasing the mitochondrial membrane potential (MMP). PMID: 23178449
    16. ECHS1 specifically represses STAT3 activity and negatively regulates the expression of several target genes of STAT3 through inhibiting STAT3 phosphorylation. PMID: 23416296
    17. Expression of mitochondrial short chain enoyl-CoA hydratase (ECHS)was significantly down-modulated in virus infected glioblastoma cells and ECHS knockdown (siRNA) impaired virus replication and cytopathic effects. PMID: 17395278

    顯示更多

    收起更多

  • 相關疾病:
    Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (ECHS1D)
  • 亞細胞定位:
    Mitochondrion matrix.
  • 蛋白家族:
    Enoyl-CoA hydratase/isomerase family
  • 組織特異性:
    Liver, fibroblast, muscle. Barely detectable in spleen and kidney.
  • 數據庫鏈接:

    HGNC: 3151

    OMIM: 602292

    KEGG: hsa:1892

    STRING: 9606.ENSP00000357535

    UniGene: Hs.76394



主站蜘蛛池模板: 亚洲 中文字幕 日韩 无码| 国产亚洲精品久久久ai换脸| 国产亚洲日韩一区二区三区| 成人自慰女黄网站免费大全| 国产99视频精品免费视看6| 天天躁日日躁狠狠躁av麻豆男男| 国产精品538一区二区在线| 国产公妇仑乱在线观看| 中文字字幕在线精品乱码| 亚洲人成网站色www| 老熟女高潮一区二区三区| 1000部精品久久久久久久久| 国产日产欧产精品精品免费| 午夜亚洲影院在线观看| 日本在线视频www鲁啊鲁| 国产激情久久久久久熟女老人| 24小时日本在线www免费的 | 精品人妻无码专区中文字幕| 国产精品一区二区久久乐下载| 麻婆豆传媒一区二区三区| 国产18禁黄网站免费观看| 日韩欧美一区二区三区免费观看| 中国熟妇牲交视频| 女人高潮抽搐潮喷视频开腿| 国产在线精品一区二区三区| 亚洲国产精品无码一线岛国| 蜜桃av无码免费看永久| 欧美 日本 国产 在线a∨观看| 麻豆传传媒久久久爱| 国产无遮挡a片又黄又爽| 日本人又黄又爽又色的视频| 中文字幕乱码熟妇五十中出色欲| 久久偷看各类wc女厕嘘嘘偷窃| 色五月五月丁香亚洲综合网| 2020精品国产视| 亚洲人成无码网站在线观看| 久久久久99精品成人片直播| 天天爱天天做天天爽夜夜揉| 岛国无码av不卡一区二区| 黑人巨大精品欧美一区二区| 午夜时刻免费入口|