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Rabbit Anti-CPOX/Cy5.5 Conjugated antibody (bs-13980R-Cy5.5)
~~~促销,代码KXJ230206-6~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-13980R-Cy5.5
英文名称 Anti-CPOX/Cy5.5
中文名称 Cy5.5标记的粪生素氧化酶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.  
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  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 (predicted: Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, Sheep, )
产品应用 ICC=1:50-200 IF=1:50-200 
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/ml
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.
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.

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.
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