PPOX
PPOX (protoporphyrinogen oxidase, also known as PPO or VP) is a flavoprotein oxidoreductase of the inner mitochondrial membrane. It catalyses the six-electron oxidation of protoporphyrinogen IX to protoporphyrin IX, the penultimate step of heme biosynthesis. Browse Assay Genie's current range of PPOX research reagents below, or read on for the biology behind the target.
PPOX is the enzyme that catalyses the six-electron oxidation of protoporphyrinogen IX to form protoporphyrin IX. This reaction is the penultimate step of the heme biosynthesis pathway, so the activity of PPOX sits close to the end of the route that produces the porphyrin ring of heme. The enzyme is assigned EC 1.3.3.4 and is classed by HGNC among the flavoproteins and the oxidoreductases.
The protein is located on the inner mitochondrial membrane. UniProt places it in the protoporphyrinogen/coproporphyrinogen oxidase family, within the protoporphyrinogen oxidase subfamily. The human PPOX gene encodes this enzyme, and the NCBI Gene summary notes that alternatively spliced transcript variants have been identified which all encode the same protein. The mouse ortholog is written Ppox, which allows comparative work on the enzyme in a widely used model organism.
The gene is also known by the aliases PPO and VP, and its previous HGNC name was variegate porphyria, which reflects its main disease association. Mutations in the PPOX gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism that results from a deficiency in protoporphyrinogen oxidase. For this reason the protein is a central subject in studies of porphyrin metabolism, mitochondrial enzymology and inherited disorders of the heme pathway.
Researchers working on PPOX typically examine its expression, its abundance in mitochondrial preparations and its relationship to other enzymes of heme biosynthesis, using the symbol, the full name protoporphyrinogen oxidase or the aliases above when searching the literature.
PPOX catalyses the penultimate step of heme biosynthesis, converting protoporphyrinogen IX to protoporphyrin IX, so it is a key enzyme for studying pathway flux.
Mutations in the PPOX gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism arising from protoporphyrinogen oxidase deficiency.
As an enzyme of the inner mitochondrial membrane, PPOX is relevant to work on how mitochondria host and organise steps of heme production.
PPOX is a flavoprotein oxidoreductase of the protoporphyrinogen/coproporphyrinogen oxidase family, making it a model for six-electron oxidation chemistry.
PPOX catalyses the six-electron oxidation of protoporphyrinogen IX to protoporphyrin IX. This is the penultimate enzymatic step of heme biosynthesis. The enzyme sits on the inner mitochondrial membrane and is classified as EC 1.3.3.4, a flavoprotein oxidoreductase.
PPOX is also called PPO and VP, and its full name is protoporphyrinogen oxidase. HGNC lists variegate porphyria as a previous name, reflecting the disease linked to the gene. Alternatively spliced transcript variants have been described, and they encode the same protein.
Mutations in the PPOX gene cause variegate porphyria, an autosomal dominant disorder of heme metabolism due to protoporphyrinogen oxidase deficiency. A mouse ortholog, Ppox, exists; check the datasheet of any product for its validated reactivity.
Other heme biosynthesis enzymes and porphyrin metabolism targets that may be studied alongside PPOX.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P50336; product specifications from Assay Genie datasheets.