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protoporphyrinogen oxidase; PPO (PPOX)

Function: - catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX - porphyrin metabolism; protoporphyrin-IX biosynthesis - protoporphyrin-IX from protoporphyrinogen-IX: step 1/1 Cofactor: - binds 1 FAD per dimer protoporphyrinogen-IX + 3 O2 = protoporphyrin-IX + 3 H2O2 Structure: - homodimer - belongs to the protoporphyrinogen oxidase family Compartment: - mitochondrial inner membrane; intermembrane side (putative) Expression: - expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney & pancreas Pathology: - defects in PPOX are the cause of variegate porphyria

Interactions

molecular events

Related

heme synthesis

General

mitochondrial protein oxidoreductase

Properties

SIZE: entity length = 477 aa MW = 51 kD COMPARTMENT: mitochondria MOTIF: cofactor-binding site [9-14] COFACTOR-BOUND: flavin adenine dinucleotide

Database Correlations

OMIM correlations MORBIDMAP 600923 UniProt P50336 Pfam PF01593 Entrez Gene 5498 Kegg hsa:5498 ENZYME 1.3.3.4

References

  1. UniProt :accession P50336
  2. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/PPOX
  3. Wikipedia; Note: protoporphyrinogen oxidase entry http://en.wikipedia.org/wiki/protoporphyrinogen_oxidase