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Rabbit Anti-PPOX Recombinant Antibody (CBYC-P559) (CBMAB-P2577-YC)

Provided herein is a Rabbit monoclonal antibody against Human Protoporphyrinogen Oxidase. The antibody can be used for immunoassay techniques, such as WB, FC, ICC, IF.
See all PPOX antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
CBYC-P559
Antibody Isotype
IgG
Application
WB, FC, ICC, IF

Basic Information

Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
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!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
protoporphyrinogen oxidase
Introduction
PPOX is 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.
Entrez Gene ID
Human5498
Mouse19044
Rat289219
UniProt ID
HumanP50336
MouseP51175
RatD3ZVN7
Alternative Names
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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