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ACO2 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA218127A0HU
  • 规格：
    ￥1320
  • 图片：
    • IHC image of CSB-RA218127A0HU diluted at 1:100 and staining in paraffin-embedded human lung cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA218127A0HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
  • 其他：

产品详情

  • 产品描述：

    ACO2 is a mitochondrial protein that catalyzes the conversion of citrate to isocitrate within the tricarboxylic acid cycle (TCA). It is critically involved in the function of the tricarboxylic acid cycle (TCA), the maintenance of iron homeostasis, oxidative stress defense, and the integrity of mitochondrial DNA (mtDNA). Mutations in the ACO2 gene were identified in patients suffering from a broad range of symptoms, including optic nerve atrophy, cortical atrophy, cerebellar atrophy, hypotonia, seizures, and intellectual disabilities. Due to its crucial function in central metabolic pathways, ACO2 is reported to play a role in various metabolic diseases such as diabetes or oncological ailments, as well as neurodegenerative diseases.

    Compared with the polyclonal and monoclonal antibodies of ACO2, this ACO2 recombinant antibody has the features of increased reproducibility and control, animal-free technology, high degree of monovalency, high batch-to-batch consistency, easier isotype conversion, etc. And it has been validated in ELISA, IHC.

  • Uniprot No.：
    Q99798
  • 基因名：
    ACO2
  • 别名：
    Aconitate hydratase, mitochondrial (Aconitase) (EC 4.2.1.3) (Citrate hydro-lyase), ACO2
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human Aconitase 2
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    8H4
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:50-1:200
  • 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：
    Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.
  • 基因功能参考文献：
    1. Aco2 activity correlated significantly with motor score, independence scale, and functional capacity of the Unified Huntington's Disease Rating Scale as well as disease duration. Our study provides a potential biomarker to assess the disease status of HD patients and PreHD carriers. PMID: 29160844
    2. Results suggest that ACO2 activity is reduced in peripheral lymphocytes of subjects with Alzheimer's disease and mild cognitive impairment and correlates with antioxidant protection PMID: 25322927
    3. Our study shows that autosomal recessive ACO2 mutations can cause either isolated or syndromic optic neuropathy. PMID: 25351951
    4. immunofluorescence staining localized ACO2 to the human sperm mid-piece. By immunoblotting, we demonstrated that the level of ACO2 protein in asthenozoospermic samples was significantly decreased compared with that in normal fertile men PMID: 24785945
    5. Ogg1 chaperoning of Aco-2 in preventing oxidant-mediated mtDNA damage and apoptosis may afford an innovative target for the molecular events underlying oxidant-induced toxicity. PMID: 24429287
    6. Hypoxia upregulates the gene expression of mitochondrial aconitase in prostate carcinoma cells PMID: 23709747
    7. Gastric cancer patients with lower ACO2 expression have a shorter survival time than those with higher ACO2 expression. PMID: 23550275
    8. Homozygosity mapping followed by whole-exome sequencing disclosed a Ser112Arg mutation in ACO2. PMID: 22405087
    9. these results suggest that p53 downregulation of mACON gene expression in human prostate carcinoma cells may not occur through the putative consensus p53 response elements found within the mACON promoter. PMID: 20607720
    10. abolishes oxidant-induced apoptosis PMID: 19524665
    11. Lon protease selectively recognizes and degrades the oxidized, hydrophobic form of aconitase after mild oxidative modification, but that severe oxidation results in aconitase aggregation, which makes it a poor substrate for Lon. PMID: 12198491
    12. ACO2 is often deleted in colorectal cancer but is unlikely to be the true target of the deletions PMID: 12746427
    13. The m-aconitase promoter is contained in a 153-bp 5' fragment lacking a TATA or CAAT sequence. Sp1 binding to specific Sp1 site is needed for promoter activity. Other transcription factors are recruited through protein-protein interactions. PMID: 16598741
    14. Manganese acts as an antagonist of iron, disrupting the enzymatic activity and gene expression of mACON and citrate metabolism in the prostate. PMID: 16625280
    15. This protein has been found differentially expressed in the Wernicke's Area from patients with schizophrenia. PMID: 19405953
    16. This protein has been found differentially expressed in the dorsolateral prefrontal cortex from patients with schizophrenia. PMID: 19110265

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  • 相关疾病：
    Infantile cerebellar-retinal degeneration (ICRD); Optic atrophy 9 (OPA9)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    Aconitase/IPM isomerase family
  • 数据库链接：

    HGNC: 118

    OMIM: 100850

    KEGG: hsa:50

    STRING: 9606.ENSP00000216254

    UniGene: Hs.643610