| 產(chǎn)品編號 | bs-13984R-FITC |
| 英文名稱1 | Rabbit Anti-COQ2/FITC Conjugated antibody |
| 中文名稱 | FITC標(biāo)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(xì)胞生物 信號轉(zhuǎn)導(dǎo) 細(xì)胞類型標(biāo)志物 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, ) |
| 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. |
