| 产品编号 | bs-5094R-AP |
| 英文名称 | Anti-UROD/AP |
| 中文名称 | 碱性磷酸酶（AP）标记的尿卟啉原脱羧酶抗体 |
| 别 名 | PCT; UPD; URO D; Uroporphyrinogen decarboxylase; Uroporphyrinogen III decarboxylase. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human, Mouse, Rat, Dog, Horse, ) |
| 产品应用 | WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 41kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human UROD |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | 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. |
| 产品介绍 |
background: UROD is the fifth enzyme of the heme biosynthetic pathway. This enzyme is responsible for catalyzing the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains. Mutations and deficiency in this enzyme are known to cause familial porphyria cutanea tarda and hepatoerythropoetic porphyria. Porphyria cutanea tarda is an autosomal dominant disorder characterized by light-sensitive dermatitis and associated with the excretion of large amounts of uroporphyrin in urine. Hepatoerythropoetic porphyria is a form of porphyria cutanae tarda that may also be a manifestation of benign or malignant hepatic tumors. Function: Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III. Subunit: Homodimer Subcellular Location: Cytoplasm. DISEASE: Defects in UROD are the cause of familial porphyria cutanea tarda (FPCT) [MIM:176100]; also known as porphyria cutanea tarda type II. FPCT is an autosomal dominant disorder characterized by light-sensitive dermatitis, with onset in later life. It is associated with the excretion of large amounts of uroporphyrin in the urine. Iron overload is often present in association with varying degrees of liver damage. Besides the familial form of PCT, a relatively common idiosyncratic form is known in which only the liver enzyme is reduced. This form is referred to as porphyria cutanea tarda "sporadic" type or type I [MIM:176090]. PCT type I occurs sporadically as an unusual accompaniment of common hepatic disorders such as alcohol-associated liver disease. Defects in UROD are the cause of hepatoerythropoietic porphyria (HEP) [MIM:176100]. HEP is a rare autosomal recessive disorder. It is the severe form of cutaneous porphyria, and presents in infancy. The level of UROD is very low in erythrocytes and cultured skin fibroblasts, suggesting that HEP is the homozygous state for porphyria cutanea tarda. Similarity: Belongs to the uroporphyrinogen decarboxylase family. Database links: Entrez Gene: 7389 Human Entrez Gene: 22275 Mouse Entrez Gene: 497117 Sheep Omim: 613521 Human SwissProt: P06132 Human SwissProt: P70697 Mouse SwissProt: Q8HY31 Sheep Unigene: 78601 Human Unigene: 46484 Mouse Unigene: 4214 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. UROD是一种参与生成血红素分子的酶（血红素-血红素蛋白,主要在血液、骨髓和肝脏中最丰富）。阻断UROD基因的表达-减低血红素蛋白在肿瘤增殖的作用-增加癌细胞的死亡。经研究认为:肿瘤中的UROD浓度比正常组织显著要高,希望UROD抑制剂的出现, 对肿瘤患者的治疗产生一定的作用。 |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
