| 產(chǎn)品編號(hào) | bs-9068R-Cy7 |
| 英文名稱1 | Rabbit Anti-HMBS/Cy7 Conjugated antibody |
| 中文名稱 | Cy7標(biāo)記的卟膽原脫氨酶抗體 |
| 別 名 | HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase. |
| 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 細(xì)胞生物 免疫學(xué) 信號(hào)轉(zhuǎn)導(dǎo) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Pig, Cow, Rabbit, Sheep, Cat, ) |
| 產(chǎn)品應(yīng)用 |
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 39kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HMBS |
| 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: This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008] Function: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Subcellular Location: Cytoplasm. Tissue Specificity: Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells. DISEASE: Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Similarity: Belongs to the HMBS family. Database links: Entrez Gene: 3145 Human Entrez Gene: 15288 Mouse Omim: 609806 Human SwissProt: P08397 Human SwissProt: P22907 Mouse Unigene: 82609 Human Unigene: 247676 Mouse Unigene: 11080 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 卟膽原脫氨酶，為血紅素等四吡咯環(huán)化合物合成通路中的催化酶。PBGD催化卟膽原(Porphobilingen，PBG)底物的線性四聚化，反應(yīng)過程中四分子卟膽原底物有序性的共價(jià)連接到酶的輔基上，最后水解形成具有四聯(lián)吡咯環(huán)結(jié)構(gòu)的尿卟啉原前體。PBGD的活力缺陷會(huì)引起急性間歇性卟啉癥(Acute Intermittant Porphyria，AIP)的發(fā)生。 |
