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Invitrogen™ PPOX Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA528374

Catalog No. PIPA528374


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Description

Description

Recommended positive controls: A549, HeLa, HCT116. Predicted reactivity: Mouse (83%), Rat (81%), Bovine (90%). Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.
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Specifications

Specifications

PPOX
Polyclonal
Unconjugated
PPOX
PPO; Ppox; protoporhyrinogen oxidase; protoporphyrinogen oxidase; V290M; VP
Rabbit
Antigen affinity chromatography
RUO
5498
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Liquid
Western Blot
0.82 mg/mL
0.1M tris glycine with 20% glycerol and 0.01% thimerosal; pH 7
P50336
PPOX
Recombinant fragment corresponding to a region within amino acids 179 and 418 of Human PPOX.
100 μL
Primary
Human
Antibody
IgG
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