| 產(chǎn)品編號(hào) | bs-9521R-BF647 |
| 英文名稱 | Rabbit Anti-FECH/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的鐵螯合酶抗體 |
| 別 名 | EPP; FCE; Ferrochelatase (protoporphyria); Ferrochelatase; Ferrochelatase mitochondrial; Heme synthetase; Protoheme ferro lyase; HEMH_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 心血管 細(xì)胞生物 免疫學(xué) 細(xì)胞類型標(biāo)志物 細(xì)胞骨架 線粒體 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, ) |
| 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 | 43kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EPB41 |
| 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: Ferrochelatase catalyzes the insertion of the ferrous form of iron into protoporphyrin IX in the heme synthesis pathway, and is localised in the mitochondrion. Defects in ferrochelatase are associated with protoporphyria. Two transcript variants encoding different isoforms have been found for this gene. Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors). They manifest with either skin problems, or neurological complications, or occasionally both. Function: Catalyzes the ferrous insertion into protoporphyrin IX. Subunit: Homodimer. Subcellular Location: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. DISEASE: Defects in FECH are the cause of erythropoietic protoporphyria (EPP) [MIM:177000]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals. Similarity: Belongs to the ferrochelatase family. Database links: Entrez Gene: 2235 Human Omim: 612386 Human SwissProt: P22830 Human Unigene: 365365 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
