产品中心
当前位置：首页>产品中心Anti-UROD/Cy7
货号： bs-5094R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5094R-Cy7
- 英文名称
- Anti-UROD/Cy7
- 中文名称
- Cy7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
- Human, Mouse, Rat, Dog, Horse,
- 产品应用
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: 7389Human
Entrez Gene: 504914Cow
Entrez Gene: 475378Dog
Entrez Gene: 22275Mouse
Entrez Gene: 29421Rat
Entrez Gene: 497117Sheep
Omim: 613521Human
SwissProt: P06132Human
SwissProt: P70697Mouse
SwissProt: P32362Rat
SwissProt: Q8HY31Sheep
Unigene: 78601Human
Unigene: 46484Mouse
Unigene: 4214Rat
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。