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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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Product Code. 16883744

  • 1656.73 SEK / 20µL
Estimated Shipment: 03-04-2024
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Description

Description

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 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.
Specifications

Specifications

PPOX
Polyclonal
Unconjugated
PPOX
PPO, PPOX, protoporphyrinogen oxidase, V290M, VP
Rabbit
Antigen Affinity Chromatography
RUO
19044, 289219, 5498
-20°C
Liquid
Western Blot, Immunohistochemistry (Paraffin)
0.15 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
P50336, P51175
Ppox
PPOX Fusion Protein Ag6541
20 μL
Primary
Human, Rat, Mouse
Antibody
IgG
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