| 產(chǎn)品編號(hào) | bs-2373R-BF594 |
| 英文名稱 | Rabbit Anti-Ceruloplasmin/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的銅藍(lán)蛋白抗體 |
| 別 名 | CER; caeruloplasmin; Ceruloplasmin precursor; CP 2; CP; CP2; CP-2; EC 1.16.3.1; Ferroxidase; CERU_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 心血管 細(xì)胞生物 免疫學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Rabbit, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 120kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Ceruloplasmin |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 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: Caeruloplasmin is a copper binding plasma glycoprotein consisting of a single polypeptide chain. It has a molecular weight of 132kDa and occurs in plasma at a concentration of 150-500 mg/L. Although the physiological functions of Caeruloplasmin are not fully understood it is implicated in a number of possible functions. 95% of plasma copper is bound to Caeruloplasmin, implying some involvement in copper transport. Caeruloplasmin has been described as both an oxidant and an antioxidant, its exact role or roles in this respect are not clear. Plasma levels of Caeruloplasmin are elevated after inflammation and trauma and it is therefore classified as an acute phase protein. The measurement of Caeruloplasmin in plasma is useful in the diagnosis of Wilson’s disease, where levels are greatly reduced. Caeruloplasmin is extremely labile in plasma and rapidly degrades to 115kDa and 19kDa fragments. The age of plasma samples should therefore be taken into account when interpreting immunological analysis of caeruloplasmin. Function: Ceruloplasmin is a blue, copper-binding (6-7 atoms per molecule) glycoprotein. It has ferroxidase activity oxidizing Fe(2+) to Fe(3+) without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu(2+) ions for the ascorbate-mediated deaminase degradation of the heparan sulfate chains of GPC1. May also play a role in fetal lung development or pulmonary antioxidant defense. Subcellular Location: Secreted. Note=Colocalizes with GCP1 in secretory intracellular compartments. Tissue Specificity: Expressed by the liver and secreted in plasma. DISEASE: Aceruloplasminemia (ACERULOP) [MIM:604290]: An autosomal recessive disorder of iron metabolism characterized by iron accumulation in the brain as well as visceral organs. Clinical features consist of the triad of retinal degeneration, diabetes mellitus and neurological disturbances. Note=The disease is caused by mutations affecting the gene represented in this entry. Note=Ceruloplasmin levels are decreased in Wilson disease, in which copper cannot be incorporated into ceruloplasmin in liver because of defects in the copper-transporting ATPase 2. Similarity: Belongs to the multicopper oxidase family. Contains 3 F5/8 type A domains. Contains 6 plastocyanin-like domains. Database links: Entrez Gene: 1356 Human Entrez Gene: 12870 Mouse Omim: 117700 Human SwissProt: P00450 Human SwissProt: Q61147 Mouse Unigene: 558314 Human Unigene: 13787 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. ????銅藍(lán)蛋白（ceruloplasmin,CER）又稱銅氧化酶，是一種含銅的α2糖蛋白，CER具有氧化酶的活性，起著抗氧化劑的作用。在血循環(huán)中CER的抗氧化活力可以防止組織中脂質(zhì)過氧化物和自由基的生成，特別在炎癥時(shí)具有重要意義,也屬于一種急性時(shí)相反應(yīng)蛋白。 ????銅藍(lán)蛋白對(duì)多酚及多胺類底物有催化其氧化之能力銅藍(lán)蛋白具有遺傳上的基因多形性。其作用為調(diào)節(jié)銅在機(jī)體各個(gè)部位的分布、合成含銅的酶蛋白.一般認(rèn)為銅藍(lán)蛋白由肝臟合成，一部分由膽道排泄，尿中含量甚微。銅藍(lán)蛋白測定對(duì)某些肝、膽、腎等疾病的診斷有一定意義。 |
