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PPOX Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
SDP

Catalog No. 501729792 Shop All Proteintech Group Inc Products
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Catalog No. 50-172-9792 Supplier Proteintech Group Inc Supplier No. 148701AP150UL
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Rabbit Polyclonal Antibody

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

Antigen PPOX
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.15 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene PPOX
Gene Accession No. P50336, P51175
Gene Alias PPO, PPOX, protoporphyrinogen oxidase, V290M, VP
Gene Symbols Ppox
Host Species Rabbit
Immunogen PPOX Fusion Protein Ag6541
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 19044, 289219, 5498
Target Species Human, Mouse, Rat
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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