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HGD Recombinant Monoclonal Antibody

  • 中文名稱:
    HGD重組抗體
  • 貨號:
    CSB-RA695552A0HU
  • 規(guī)格:
    ¥1320
  • 圖片:
    • IHC image of CSB-RA695552A0HU diluted at 1:100 and staining in paraffin-embedded human liver 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°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA695552A0HU diluted at 1:100 and staining in paraffin-embedded human prostate 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°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品描述:
    CSB-RA695552A0HU HGD重組單克隆抗體是針對人類尿黑酸1,2-雙加氧酶(Homogentisate 1,2-dioxygenase)研發(fā)的高特異性檢測工具,該酶是酪氨酸代謝途徑中的關(guān)鍵催化酶,其功能缺陷與遺傳性尿黑酸癥及相關(guān)骨關(guān)節(jié)病變密切相關(guān)。本抗體經(jīng)ELISA和免疫組化(IHC)嚴(yán)格驗(yàn)證,在1:50-1:200稀釋范圍內(nèi)可清晰顯示組織樣本中HGD蛋白的定位分布,實(shí)驗(yàn)數(shù)據(jù)顯示其在多種人類組織切片中呈現(xiàn)特異性染色模式,檢測結(jié)果與靶蛋白預(yù)期表達(dá)特征高度吻合,且不同批次產(chǎn)品具有良好穩(wěn)定性。適用于探究HGD在代謝性疾病發(fā)生發(fā)展中的作用機(jī)制,特別是在構(gòu)建酪氨酸代謝異常模型、骨關(guān)節(jié)退行性病變研究及病理樣本分析等領(lǐng)域具有重要應(yīng)用價值。該抗體為科研人員研究HGD蛋白表達(dá)調(diào)控及其在遺傳代謝缺陷中的病理生理意義提供了可靠工具。
  • Uniprot No.:
  • 基因名:
    HGD
  • 別名:
    Homogentisate 1,2-dioxygenase (EC 1.13.11.5) (Homogentisate oxygenase) (Homogentisic acid oxidase) (Homogentisicase), HGD, HGO
  • 反應(yīng)種屬:
    Human
  • 免疫原:
    A synthesized peptide derived from human HGD
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Non-conjugated
  • 克隆類型:
    Monoclonal
  • 抗體亞型:
    Rabbit IgG
  • 純化方式:
    Affinity-chromatography
  • 克隆號:
    20D12
  • 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)品評價

靶點(diǎn)詳情

  • 基因功能參考文獻(xiàn):
    1. Twelve novel HGD gene variants have been identified in 99 alkaptonuria patients affecting the bones. PMID: 25804398
    2. A novel alkaptonuria mutation, c.87 + 1G > A, shows a significant founder effect and high prevalence in a nomadic Indian population. PMID: 24575791
    3. study described two novel HGD mutations in a Chinese alkaptonuria family, the splicing mutation of IVS7 1G>C, a donor splice site of exon 7, and a missense mutation of F329C in exon 12 PMID: 23353776
    4. Report mutations of the HGD gene in Jordanian alkaptonuria patients. PMID: 21437689
    5. The observed increase of HGD expression in Alkaptonuria cells is probably due to a compensatory mechanism to overcome the almost null catalytic activity of the deficient enzyme PMID: 22105303
    6. An update on molecular genetics of Alkaptonuria (AKU).(review) PMID: 21720873
    7. Familiar ochronotic arthropathy is caused by a gene mutation of the homogentisic acid 1,2-dioxygenase (HGD) gene traced three hundred years in a Hungarian family. PMID: 20462779
    8. A comprehensive mutation analysis of 93 patients enrolled in this study, as well as an extensive update of all previously published HGD mutations associated with alkaptonuria, is reported. PMID: 19862842
    9. Turkish mutation shares an homogentisate 1,2-dioxygenase haplotype with the mutation found in Finland, Slovakia and India, suggesting that R58fs is an old alkaptonuria mutation that probably originated in central Asia PMID: 12872836
    10. Four different mutations of the HGD gene were found in alkaptonuria and ochronotic arthropathy diagnoses. PMID: 16085442
    11. A single nucleotide deletion located in exon 3 resulted in a frameshift mutation in HGD gene in family with alkaptonuria. PMID: 18945288

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  • 相關(guān)疾病:
    Alkaptonuria (AKU)
  • 蛋白家族:
    Homogentisate dioxygenase family
  • 組織特異性:
    Highest expression in the prostate, small intestine, colon, kidney and liver.
  • 數(shù)據(jù)庫鏈接:

    HGNC: 4892

    OMIM: 203500

    KEGG: hsa:3081

    STRING: 9606.ENSP00000283871

    UniGene: Hs.368254