BackgroundThis 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.
DescriptionPPOX Polyclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
SpecificityHuman, Mouse
IsotypeIgG
Uniprot IDP50336
PurificationAffinity Purified
ImmunogenRecombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human PPOX (NP_001116236.1).
StorageShipped at 4°C. Upon receipt, store at -20°C. Avoid freeze / thaw cycles
Alternative NamesPPOX; PPO; V290M; VP; protoporphyrinogen oxidase
ApplicationWB, IHC