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PPOX Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
Brand: Proteintech 14870-1-AP-20UL
Additional Details : Weight : 0.00010kg
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DescriptionProtoporphyrinogen 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 domit 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.
|PPO, PPOX, protoporphyrinogen oxidase, V290M, VP|
|Antigen Affinity Chromatography|
|19044, 289219, 5498|
|Immunohistochemistry (Paraffin), Western Blot|
|PBS with 50% glycerol and 0.02% sodium azide; pH 7.3|
|PPOX Fusion Protein Ag6541|
|Mouse, Rat, Human|