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

More general terms

• oxidoreductase
• mitochondrial protein

Additional terms

• heme synthesis

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5498
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5498
• OMIM: https://mirror.omim.org/entry/176200
• OMIM: https://mirror.omim.org/entry/600923
• UniProt: http://www.uniprot.org/uniprot/P50336.html