PPOX Antibody (PACO20270)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO20270
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | PPOX Antibody (PACO20270) |
Antibody SKU: | PACO20270 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:5000, IHC:1:50-1:200 |
Species Reactivity: | Human, Mouse |
Immunogen: | Synthetic peptide of human PPOX |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
The image on the left is immunohistochemistry of paraffin-embedded Human liver cancer tissue using PACO20270(PPOX Antibody) at dilution 1/40, on the right is treated with synthetic peptide. (Original magnification: x200). |
Background: | This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified. |
Synonyms: | protoporphyrinogen oxidase |
UniProt Protein Function: | PPOX: Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX. Defects in PPOX are the cause of variegate porphyria (VP). Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. PV is the most common form of porphyria in South Africa. It is characterized by skin hyperpigmentation and hypertrichosis, abdominal pain, tachycardia, hypertension and neuromuscular disturbances. High fecal levels of protoporphyrin and coproporphyrin, increased urine uroporphyrins and iron overload are typical markers of the disease. Belongs to the protoporphyrinogen oxidase family. |
UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Oxidoreductase; Mitochondrial; EC 1.3.3.4 Chromosomal Location of Human Ortholog: 1q22 Cellular Component: mitochondrial membrane; mitochondrial intermembrane space; integral to mitochondrial inner membrane; intrinsic to mitochondrial inner membrane Molecular Function:protoporphyrinogen oxidase activity; FAD binding Biological Process: response to drug; porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; porphyrin biosynthetic process; heme biosynthetic process Disease: Porphyria Variegata |
NCBI Summary: | This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008] |
UniProt Code: | P50336 |
NCBI GenInfo Identifier: | 1709742 |
NCBI Gene ID: | 5498 |
NCBI Accession: | P50336.1 |
UniProt Secondary Accession: | P50336,Q5VTW8, D3DVG0, |
UniProt Related Accession: | P50336 |
Molecular Weight: | 477 |
NCBI Full Name: | Protoporphyrinogen oxidase |
NCBI Synonym Full Names: | protoporphyrinogen oxidase |
NCBI Official Symbol: | PPOX |
NCBI Official Synonym Symbols: | VP; PPO; V290M |
NCBI Protein Information: | protoporphyrinogen oxidase |
UniProt Protein Name: | Protoporphyrinogen oxidase |
UniProt Gene Name: | PPOX |
UniProt Entry Name: | PPOX_HUMAN |
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