產(chǎn)品編號 | bs-13984R-BF594 |
英文名稱 | Rabbit Anti-COQ2/BF594 Conjugated antibody |
中文名稱 | BF594標記的線粒體COQ2抗體 |
別 名 | 2310002F18Rik; 4-hydroxybenzoate polyprenyltransferase; CL640; Coenzyme Q2 homolog, prenyltransferase (yeast); COQ2; COQ2 homolog; COQ2_HUMAN; FLJ13014; FLJ26072; hCOQ2; MGC124824; MGC91278; mitochondrial; OTTMUSP00000032111; Para-hydroxybenzoate--polyprenyltransferase; Para-hydroxybenzoate--polyprenyltransferase, mitochondrial; PHB:polyprenyltransferase; RGD1306722. |
規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
說 明 書 | 100ul |
研究領(lǐng)域 | 腫瘤 細胞生物 信號轉(zhuǎn)導(dǎo) 細胞類型標志物 線粒體 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | |
產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 37kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human COQ2 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
產(chǎn)品介紹 |
background: This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009] Function: Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB. Subcellular Location: Mitochondrion membrane. Tissue Specificity: Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart. DISEASE: Defects in COQ2 are the cause of coenzyme Q10 deficiency, primary, type 1 (COQ10D1) [MIM:607426]. An autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. Similarity: Belongs to the UbiA prenyltransferase family. Database links: Entrez Gene: 27235 Human Omim: 609825 Human SwissProt: Q96H96 Human Unigene: 144304 Human Unigene: 729069 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
1、抗體溶解方法 | |
2、抗體修復(fù)方式 | |
3、常用試劑的配制 | |
4、免疫組化操作步驟 | |
5、免疫組化問題解答 | |
6、Western Blotting 操作步驟 | |
7、Western Blotting 問題解答 | |
8、關(guān)于肽鏈的設(shè)計 | |
9、多肽的溶解與保存 | |
10、酶標抗體效價測定程序 | |
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