| Protein Name | protoporphyrinogen oxidase |
|---|---|
| Organism | Homo sapiens |
| Gene ID | 5498 |
| Gene Symbol | PPOX |
| UniProt | P50336 (PPOX_HUMAN) |
| Relationships |
Total Number of functionally related compound(s) :
531
Total Number of Articles : 456 |
Description |
protoporphyrinogen oxidase |
| Gene Summary |
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] |
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