Invitrogen™ PPOX Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA556353

Product Code. 15784261

Description

Immunogen sequence: ETHLTSRGVS VLRGQPVCGL SLQAEGRWKV SLRDSSLEAD HVISAIPASV LSELLPAEAA PLARALSAIT AVSVAVVNLQ YQGAH Highest antigen sequence identity to the following orthologs: Mouse - 81%, Rat - 76%.

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.

Specifications

• Antigen: PPOX
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: PPOX
• Gene Alias: PPO; Ppox; protoporhyrinogen oxidase; protoporphyrinogen oxidase; V290M; VP
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 5498
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Immunocytochemistry
• Concentration: 0.2 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: P50336
• Gene Symbols: PPOX
• Immunogen: Recombinant protein corresponding to Human PPOX. Recombinant protein control fragment (Product #RP-94274).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG