PPOX, Polyclonal Antibody

VP; PPO; V290M

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-PPOX antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB: 1:500 - 1:2000
IHC: 1:50 - 1:200
IF: 1:50 - 1:100

Western blot analysis of extracts of various cell lines, using PPOX Antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 90s.

Immunohistochemistry of paraffin-embedded human colon carcinoma using PPOX antibody at dilution of 1:100 (40x lens).

Immunohistochemistry of paraffin-embedded human liver injury using PPOX antibody at dilution of 1:100 (40x lens).

Immunohistochemistry of paraffin-embedded human lung cancer using PPOX antibody at dilution of 1:100 (40x lens).

Immunohistochemistry of paraffin-embedded human stomach using PPOX antibody at dilution of 1:100 (40x lens).

477

protoporphyrinogen oxidase

protoporphyrinogen oxidase

Protoporphyrinogen oxidase

PPO  [Similar Products]

PPOX_HUMAN

This gene encodes the penultimate enzyme of heme biosynthesis, which catalyzes the 6-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. Mutations in this gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008]

PPOX: Catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX. Defects in PPOX are the cause of variegate porphyria (VP). Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. PV is the most common form of porphyria in South Africa. It is characterized by skin hyperpigmentation and hypertrichosis, abdominal pain, tachycardia, hypertension and neuromuscular disturbances. High fecal levels of protoporphyrin and coproporphyrin, increased urine uroporphyrins and iron overload are typical markers of the disease. Belongs to the protoporphyrinogen oxidase family.

Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Oxidoreductase; Mitochondrial; EC 1.3.3.4

Chromosomal Location of Human Ortholog: 1q22

Cellular Component: mitochondrial membrane; mitochondrial intermembrane space; integral to mitochondrial inner membrane; intrinsic to mitochondrial inner membrane

Molecular Function: protoporphyrinogen oxidase activity; FAD binding

Biological Process: response to drug; porphyrin metabolic process; protoporphyrinogen IX biosynthetic process; porphyrin biosynthetic process; heme biosynthetic process

Disease: Porphyria Variegata

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