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Invitrogen
Human PPOX (aa 241-325) Control Fragment Recombinant Protein
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Product Details
RP-94274
Species
Expression System
Amino acid sequence
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Class
Type
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Conjugate
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Purification
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Product Specific Information
Highest antigen sequence indentity to the following orthologs: Mouse (81%), Rat (81%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-56353. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
Target Information
Protoporphyrinogen oxidase, the penultimate enzyme in the heme biosynthetic pathway, catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. The PPOX protein localizes to the inner membrane of mitochondria from various tissues, including heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Genetic deficiency of PPOX results in variegate porphyria, a low penetrance, autosomal dominant disorder characterized by cutaneous photosensitivity and/or various neurological manifestations. The rare homozygous variant of VP is characterized by severe PPOX deficiency, and results in the onset of photosensitization by porphyrins in early childhood, skeletal abnormalities of the hand, and, less constantly, short stature, mental retardation and convulsions.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
References (0)
Bioinformatics
Protein Aliases: PPO; Protoporphyrinogen oxidase
Gene Aliases: PPO; PPOX; V290M; VP
UniProt ID: (Human) P50336
Entrez Gene ID: (Human) 5498
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