CPOX Antibody

中文名稱：

CPOX兔多克隆抗體
貨號：

CSB-PA005910LA01HU
規格：

￥440
圖片：
Western Blot
Positive WB detected in: HepG2 whole cell lysate, A549 whole cell lysate
All lanes: CPOX antibody at 5.7µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 51, 31 kDa
Observed band size: 51 kDa

IHC image of CSB-PA005910LA01HU diluted at 1:300 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

Immunofluorescence staining of HepG2 cells with CSB-PA005910LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
其他：

產品詳情
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) CPOX Polyclonal antibody
Uniprot No.：

P36551
基因名：

CPOX
別名：

Coprogen oxidase antibody; COPROPORPHYRIA antibody; Coproporphyrinogen III oxidase antibody; Coproporphyrinogen Oxidase (CPOX) antibody; Coproporphyrinogen-III oxidase antibody; Coproporphyrinogenase antibody; COX antibody; CPO antibody; Cpox antibody; CPX antibody; HEM 6 antibody; Hem-6 antibody; Hem6 antibody; HEM6_HUMAN antibody; mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial protein (19-149AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，CPOX Antibody (CSB-PA005910LA01HU)，的標記方式是Non-conjugated。對于CPOX Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA005910LB01HU	CPOX Antibody, HRP conjugated	ELISA
FITC	CSB-PA005910LC01HU	CPOX Antibody, FITC conjugated
Biotin	CSB-PA005910LD01HU	CPOX Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC, IF

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:200-1:500
IF	1:50-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.
基因功能參考文獻：
1. The monomer form of mutated CPOX did not show any activity and homodimeric enzymes derived from Hereditary coproporphyria (HCP) mutant showed low activity (<20% of the control). PMID: 24078084
2. Polymorphism of coproporphyrinogen oxidase is associated with genetic susceptibility to the adverse neurobehavioral effects of Hg exposure in children. PMID: 22765978
3. Deletion of the fifth exon in the CPOX gene is associated with hereditary coproporphyria. PMID: 21231929
4. CPOX polymorphisms are associated with biological media contamination and apoptosis disorders. PMID: 22288185
5. competitive action of both uroporphyrinogen decarboxylase and CPO on the same diacetate porphyrinogen substrate provides additional perspectives on the potential existence of abnormal pathways for heme biosynthesis PMID: 21277781
6. disease-producing mutations in the CPO gene in nine Swedish families with hereditary coproporphyria PMID: 12181641
7. coproporphyrinogen III oxidase sequence matches many structural features from urate oxidase PMID: 12208494
8. Modulation of penetrance by the wild-type allele in dominantly inherited erythrohepatic and acute hepatic porphyrias was studied using CPO. PMID: 14669009
9. All other type of mutations or missense mutations mapped elsewhere throughout the CPO gene, lead to coproporphyrin accumulation and subsequently typical HCP. PMID: 16159891
10. CPO mutations form the structural basis of hereditary coproporphyria. PMID: 16176984
11. The Km recognition) and Kcat values for coproporphyrinogen III and IV were determined. PMID: 16258391
12. His158 of human CPO may have a role in the active site, but none of the conserved histidine residues of human coproporphyrinogen oxidase is essential for catalytic activity. PMID: 17179900
13. The authors report the association of a novel mutation in the coproporphyrinogen oxidase gene in an Irish pedigree with the devlopment of hereditary coproporphyria PMID: 18557518
14. Three of the novel missense mutations and one frameshift mutation was detected in coproporphyrinogen III oxidase (CPO) gene in five Italian patients affected by Hereditary Coproporphyria (HCP). PMID: 19267996
15. biochemical & kinetic properties of CPOX4, the product of a polymorphism of the CPOX gene that modifies effects of mercury on neurobehavioral function; suggests CPOX4 may predispose to impaired heme biosynthesis which is limited further by Hg exposure PMID: 19339664
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相關疾?。?/div>
Hereditary coproporphyria (HCP)
亞細胞定位：

Mitochondrion intermembrane space.
蛋白家族：

Aerobic coproporphyrinogen-III oxidase family
數據庫鏈接：

HGNC: 2321

OMIM: 121300

KEGG: hsa:1371

STRING: 9606.ENSP00000264193

UniGene: Hs.476982

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