CPOX,粪生素氧化酶CPOX抗体

产品编号HZ-13980R
英文名称CPOX
中文名称粪生素氧化酶CPOX抗体
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; mitochondrial.
说 明 书0.1ml 0.2ml
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
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, Sheep,
CPOX,粪生素氧化酶CPOX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
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human 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.
CPOX,粪生素氧化酶CPOX抗体PubMedPubMed
产品介绍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 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

CPOX,粪生素氧化酶CPOX抗体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.