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ACO2 Recombinant Monoclonal Antibody

  • 中文名稱(chēng)：
    ACO2重組抗體
  • 貨號(hào)：
    CSB-RA218127A0HU
  • 規(guī)格：
    ￥1320
  • 圖片：
    • 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：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    CSB-RA218127A0HU ACO2重組單克隆抗體是針對(duì)線(xiàn)粒體烏頭酸酶2（ACO2）開(kāi)發(fā)的高特異性檢測(cè)試劑，適用于酶聯(lián)免疫吸附實(shí)驗(yàn)（ELISA）和免疫組織化學(xué)（IHC）分析。該抗體靶向的ACO2是線(xiàn)粒體三羧酸循環(huán)中的關(guān)鍵酶，負(fù)責(zé)催化檸檬酸與異檸檬酸之間的異構(gòu)化反應(yīng)，在細(xì)胞能量代謝和鐵硫簇組裝過(guò)程中發(fā)揮重要作用。經(jīng)實(shí)驗(yàn)驗(yàn)證，該抗體在IHC應(yīng)用中展現(xiàn)出優(yōu)異的組織特異性，推薦使用1:50-1:200稀釋比例時(shí)能清晰顯示人源樣本中ACO2蛋白的亞細(xì)胞定位，其顯色信號(hào)與線(xiàn)粒體分布特征高度吻合，背景干擾極低。在ELISA平臺(tái)中可實(shí)現(xiàn)對(duì)ACO2蛋白的定量檢測(cè)，線(xiàn)性范圍符合常規(guī)實(shí)驗(yàn)需求。本產(chǎn)品適用于能量代謝調(diào)控機(jī)制研究、線(xiàn)粒體功能障礙相關(guān)疾病模型的蛋白表達(dá)分析，以及氧化應(yīng)激條件下酶活性變化的關(guān)聯(lián)性探索等科研領(lǐng)域，為解析代謝性疾病、神經(jīng)退行性疾病及鐵代謝異常的分子機(jī)制提供可靠工具。
  • Uniprot No.：
  • 基因名：
    ACO2
  • 別名：
    Aconitate hydratase, mitochondrial (Aconitase) (EC 4.2.1.3) (Citrate hydro-lyase), ACO2
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from human Aconitase 2
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類(lèi)型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號(hào)：
    8H4
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:50-1:200
  • Protocols：
  • 儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.
  • 基因功能參考文獻(xiàn)：
    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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  • 相關(guān)疾病：
    Infantile cerebellar-retinal degeneration (ICRD); Optic atrophy 9 (OPA9)
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    Aconitase/IPM isomerase family
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 118

    OMIM: 100850

    KEGG: hsa:50

    STRING: 9606.ENSP00000216254

    UniGene: Hs.643610