COQ4 Antibody
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中文名稱：COQ4兔多克隆抗體
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貨號：CSB-PA896885LA01HU
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規格：￥440
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圖片：
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Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA896885LA01HU at dilution of 1:100 -
Immunohistochemistry of paraffin-embedded human spleen tissue using CSB-PA896885LA01HU at dilution of 1:100
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody
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Uniprot No.：
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基因名：COQ4
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別名：CGI 92 antibody; Coenzyme Q biosynthesis protein 4 homolog antibody; coq4 antibody; COQ4_HUMAN antibody; mitochondrial antibody; Ubiquinone biosynthesis protein COQ4 homolog antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial protein (31-265AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
本頁面中的產品，COQ4 Antibody (CSB-PA896885LA01HU)，的標記方式是Non-conjugated。對于COQ4 Antibody，我們還提供其他標記。見下表：
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA, IHC
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推薦稀釋比：
Application Recommended Dilution IHC 1:20-1:200 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.
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基因功能參考文獻：
- The COQ4 mutation was CRISPR/Cas9 edited resulting in isogenic, diploid and off-target free COQ4-corrected iPSCs. PMID: 28465093
- Three genes in our epilepsy cohort (COQ4, DNM1, and PURA), accounting for 14% (3/21) of all novel genetic etiologies identified in patients with epilepsy, were subsequently confirmed in independent publications. PMID: 26795593
- five recessive missense mutations in COQ4 segregating with lethal neonatal mitochondrial encephalomyopathy in four families of Ashkenzi Jews PMID: 26185144
- COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. PMID: 25658047
- Haploinsufficiency in COQ4 resulted in reduced COQ4 expression, CoQ10 content and biosynthetic rate, and activities of respiratory chain complex II+III. PMID: 22368301
- Human ortholog of Saccharomyces cerevisiae COQ4 was cloned and characterized. PMID: 18474229
- homologous to Coq4p in S.cerevisiae PMID: 11469793
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相關疾病：Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
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亞細胞定位：[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
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蛋白家族：COQ4 family
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組織特異性：Expressed ubiquitously, but at high levels in liver, lung and pancreas.
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數據庫鏈接：
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