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Rabbit Anti-CPOX  antibody (bs-13980R)  
~~~促銷代碼KT202411~~~
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說明書: 50ul  100ul  200ul
50ul/1180.00元
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200ul/2800.00元
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產品編號 bs-13980R
英文名稱 Rabbit Anti-CPOX  antibody
中文名稱 糞生素氧化酶CPOX抗體
別    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.  
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Sheep,Chicken,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 39kDa
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Coproporphyrinogen Oxidase: 351-454/454 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P36551

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



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