Coproporphyrinogen III Oxidase,原卟啉氧化酶3抗体

产品编号HZ-8860R
英文名称Coproporphyrinogen III Oxidase
中文名称原卟啉氧化酶3抗体
别 名Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN.
说 明 书0.1ml 0.2ml
研究领域细胞生物 发育生物学 干细胞 表观遗传学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep,
Coproporphyrinogen III Oxidase,原卟啉氧化酶3抗体产品应用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.
分 子 量39kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase
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.
Coproporphyrinogen III Oxidase,原卟啉氧化酶3抗体PubMedPubMed
产品介绍background:
CPOX is a 454 amino acid mitochondrial enzyme that is localized to the inner membrane space of erythrocytes. It participates in the sixth step of heme biosynthesis by catalyzing the formation of protoporphyrinogen IX from copropophyrinogen III. Mutations in the gene encoding CPOX are the cause of coproporphyria, an autosomal dominant disease characterized by skin photosensitivity and neurological disturbances. Symptoms are often experienced as attacks, which include severe abdominal and nerve pain. People affected by coproporphyria overexcrete copropophyrinogen III in feces and urine and the enzymatic activity of CPOX is found to be approximately half that of normal, leading to a decrease in overall heme synthesis. There is no cure for coproporphyria, but preventative treatment to relieve symptoms usually involves dietary changes and avoidance of drugs and alcohol.

Function:
Coproporphyrinogen Oxidase (CPOX) is the sixth enzyme of the heme biosynthetic pathway. This soluble protein is localized in the intermembrane space of mitochondria and its catalytic activy mediates the coversion of Coproporphyrinogen-III + O2 + 2 H+ into Protoporphyrinogen-IX + 2 CO2 + 2 H2O. Defects in CPOX are the cause of hereditary coproporphyria

Subunit:
Homodimer.

Subcellular Location:
Mitochondrion intermembrane space.

Coproporphyrinogen III Oxidase,原卟啉氧化酶3抗体DISEASE:
Defects in 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 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.