Reactome | PPO oxidises PPGEN9 to PRIN9

PPO oxidises PPGEN9 to PRIN9

Stable Identifier

R-HSA-189423

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial intermembrane space

Synonyms

3 O2 + protoporphyrinogen IX => 3 H2O2 + protoporphyrin IX, Oxidation of protoporphyrinogen IX to protoporphyrin IX

ReviewStatus

5/5

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Six electrons are oxidized in protophorphyrinogen IX (PPGEN9) to form the planar macrocycle protoporphyrin IX (PRIN9). This reaction is performed by the enzyme protoporphyrinogen oxidase (PPO). PPO functions as a homodimer containing one noncovalently-bound FAD. The protein resides on the outer surface of the inner mitochondrial membrane. PPO deficiency is associated with variegate porphyria in vivo (Dailey & Dailey 1996; Wang et al. 2013).

Literature References

PubMed ID	Title	Journal	Year
23467411	Quantitative structural insight into human variegate porphyria disease Wang, B, Wen, X, Qin, X, Wang, Z, Tan, Y, Shen, Y, Xi, Z	J Biol Chem	2013
8771201	Human protoporphyrinogen oxidase: expression, purification, and characterization of the cloned enzyme Dailey, TA, Dailey, HA	Protein Sci	1996