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Mouse Anti-PPOX Recombinant Antibody (1E1) (CBMAB-A6952-LY)

The product is antibody recognizes PPOX. The antibody 1E1 immunoassay techniques such as: WB, ELISA.
See all PPOX antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1E1
Antibody Isotype
IgG1, κ
Application
WB, ELISA

Basic Information

Immunogen
PPOX (AAH02357, 378 a.a. ~ 477 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
protoporphyrinogen oxidase
Introduction
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]
Entrez Gene ID
5498
UniProt ID
P50336
Alternative Names
MGC8485; PPO; V290M; VP
Function
Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
Biological Process
Heme biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Porphyrin-containing compound biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Protoporphyrinogen IX biosynthetic processIEA:UniProtKB-UniPathway
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Variegate porphyria (VP):
A form of porphyria. 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. Variegate porphyria 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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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