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ECHS1 Antibody

  • 中文名稱:
    ECHS1兔多克隆抗體
  • 貨號:
    CSB-PA007382GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • 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,Rat
  • 免疫原:
    Human ECHS1
  • 免疫原種屬:
    Homo sapiens (Human)
  • 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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IHC
  • 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

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  • 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



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