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SUCLA2 Antibody

  • 货号：
    CSB-PA868398ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: SUCLA2 antibody at 0.85µg/ml
      Lane 1: Rat heart tissue
      Lane 2: Hela whole cell lysate
      Lane 3: A549 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 51, 49 kDa
      Observed band size: 51 kDa
    • Immunohistochemistry of paraffin-embedded human ovarian cancer using CSB-PA868398ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA868398ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) SUCLA2 Polyclonal antibody
  • Uniprot No.：
    Q9P2R7
  • 基因名：
    SUCLA2
  • 别名：
    A BETA antibody; A SCS antibody; ATP specific succinyl CoA synthetase subunit beta antibody; ATP specific succinyl CoA synthetase, beta subunit antibody; ATP-specific succinyl-CoA synthetase subunit beta antibody; Mitochondrial succinyl CoA ligase [ADP forming] subunit beta antibody; MTDPS5 antibody; Renal carcinoma antigen NY-REN-39 antibody; Renal carcinoma antigen NYREN39 antibody; SCS betaA antibody; SCS-betaA antibody; SUCB1_HUMAN antibody; Succinate CoA ligase (ADP forming) antibody; Succinate CoA ligase [ADP forming] subunit beta, mitochondrial, succinyl CoA ligase [ADP forming] subunit beta, mitochondrial antibody; Succinate CoA ligase ADP forming beta subunit antibody; Succinate CoA ligase beta subunit antibody; Succinyl CoA ligase [ADP-forming] subunit beta, mitochondrial antibody; Succinyl CoA synthetase beta A chain antibody; Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial antibody; Succinyl-CoA synthetase beta-A chain antibody; SUCLA2 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Rat
  • 免疫原：
    Recombinant Human Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial protein (1-180AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit.
  • 基因功能参考文献：
    1. SUCLA2 mutation is associated with Down syndrome and mitochondrial depletion syndrome. PMID: 28749033
    2. The patient's cells lack the SUCLG1 protein, with significantly reduced levels of SUCLA2 and SUCLG2 protein PMID: 27484306
    3. Sucla2 is related to the developmental stages of mouse spermatogenesis. Knockdown of Sucla2 decreases the viability of mouse spermatocytes by inducing apoptosis via decreased mitochondrial function of the cells. PMID: 27766610
    4. SUCLA2 mutations should be analyzed in patients with slowly progressive encephalomyopathy, even in the absence of methylmalonic aciduria or mitochondrial DNA depletion. PMID: 24986829
    5. This study demonstrated that SUCLA2 is expressed exclusively in neurons in the human cerebral cortex. PMID: 24085565
    6. Identification of a novel mutation in SUCLA2 in two cousins affected with encephalomyopathy, is reported. PMID: 23759946
    7. A novel homozygous mutation in SUCLA2 gene has been associated with severe mitochondrial encephalomyopathies in two Italian siblings. PMID: 23010432
    8. X-linked sideroblastic anemia due to carboxyl-terminal ALAS2 mutations that cause loss of binding to the beta-subunit of succinyl-CoA synthetase (SUCLA2). PMID: 22740690
    9. Mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria is associated with mutations in SUCLA2, the gene encoding a beta subunit of succinate-CoA ligase. PMID: 19526370
    10. Deficiency of SUCLA2 is associated with encephalomyopathy and mitochondrial DNA depletion. PMID: 15877282

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  • 相关疾病：
    Mitochondrial DNA depletion syndrome 5 (MTDPS5)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    Succinate/malate CoA ligase beta subunit family, ATP-specific subunit beta subfamily
  • 组织特异性：
    Widely expressed. Not expressed in liver and lung.
  • 数据库链接：

    HGNC: 11448

    OMIM: 603921

    KEGG: hsa:8803

    STRING: 9606.ENSP00000367923

    UniGene: Hs.743361