粪生素氧化酶颁笔翱齿抗体
规格：1尘驳/1尘濒
英文名： CPOX
别名： Coproporphyrinogen Oxidase; Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogen-III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN
分子量： 39kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Copropor
交叉反应：Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, Sheep,
细胞定位：
粪生素氧化酶颁笔翱齿抗体产物介绍：background: The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009] Function: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. Subcellular Location: Mitochondrion intermembrane space. DISEASE: Defects in 粪生素氧化酶颁笔翱齿抗体CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or 粪生素氧化酶颁笔翱齿抗体stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity. Similarity: Belongs to the aerobic coproporphyrinogen-III oxidase family. Gene ID: 1371 Database links: Entrez Gene: 1371 Human Entrez Gene: 12892 Mouse Entrez Gene: 304024 Rat Omim: 612732 Human SwissProt: P36551 Human SwissProt: P36552 Mouse SwissProt: Q3B7D0 Rat Unigene: 476982 Human Unigene: 291519 Mouse Unigene: 19581 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
粪生素氧化酶颁笔翱齿抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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 神经生物学 信号转导 新陈代谢 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid