尿卟啉原脱羧酶抗体
规格：1mg/1ml
英文名： UROD
别名： PCT; UPD; URO D; Uroporphyrinogen decarboxylase; Uroporphyrinogen III decarboxylase.
分子量： 41kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human UROD
交叉反应：Human, Mouse, Rat, Dog, Horse,
细胞定位：细胞浆
尿卟啉原脱羧酶抗体产品介绍：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尿卟啉原脱羧酶抗体 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: 504914 Cow Entrez Gene: 475378 Dog Entrez Gene:尿卟啉原脱羧酶抗体 22275 Mouse Entrez Gene: 29421 Rat Entrez Gene: 497117 Sheep Omim: 613521 Human SwissProt: P06132 Human SwissProt: P70697 Mouse SwissProt: P32362 Rat 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*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**产生一定的作用。
尿卟啉原脱羧酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid