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unreviewed

Protoporphyrinogen oxidase (PPOX)

1 reactions · 1 pathways

Cofactors used

FAD · B2 (Riboflavin) O2

What it does, reaction by reaction

1 reactions

Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol

step 7 Irreversible

Protoporphyrinogen IX + 3 O₂ → protoporphyrin IX + 3 H₂O₂ (net oxidation)

Converts Protoporphyrinogen IX O2 into Protoporphyrin IX H2O2

› Notes

Protoporphyrinogen oxidase (PPOX), a FAD-dependent enzyme associated with the inner mitochondrial membrane, oxidizes the reduced porphyrinogen to the conjugated porphyrin. Molecular oxygen is the terminal electron acceptor; this step is essentially irreversible.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

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Europe PMC · from cache · sorted by publication date

  1. 1
    Targeted identification of new phaterpenes and elucidation of the relevant biosynthetic pathway in <i>Streptomyces phaeochromogenes</i> OSK-123.

    Fan X, Zhang X, Tang M, Wei S, Guo Z, Ren F, Hao J, Hua L, Zhou L, Xu J, Huang W, Kang Q, Bai L. · 2026-01-25

    open access unreviewed
  2. 2
    High frequency of the PPOX p.Arg168His pathogenic variant among Brazilian patients with variegate porphyria.

    Paiva MA, Covaleski APPM, Silva TO, Bittar-Braune C, Franz C, Holanda Mendonça R, Moreno CAM, Zanoteli E, Mac… · 2026-08-04

    unreviewed
  3. 3
    Challenges in Porphyria diagnosis and management: When variegate Porphyria meets HIV.

    Varela LS, Melito VA, Caballero FA, Guolo M, Goñi V, Baccaro F, Agosta M, Pasman M, Pachioli V, Tomassi VL, B… · 2026-07-30

    unreviewed
  4. 4
    Updates to gene-disease classifications and inheritance patterns for porphyrias.

    Reeves EB, Hankey W, Clair PS, Goldstein JL, Wilke MVMB, Feigenbaum A, Hung C, Spector E, Craigen WJ. · 2026-04-30

    unreviewed
  5. 5
    AI-Powered Deep Visual Proteomics Reveals Critical Molecular Transitions in Pancreatic Cancer Precursors.

    Min J, Schweizer L, Zonderland G, Selvanesan BC, Thomsen JH, Oldenburg L, Bae SW, Kim B, Hernández SD, Jez G,… · 2026-07-01

    open access unreviewed
  6. 6
    <i>SF3B1</i> <sup>K700E</sup>-driven transcriptional alterations in hematopoietic progenitors underlie blood cancer pathophysiology.

    Xue M, An J, Hu E, Deng W, Yin S. · 2025-07-15

    cited 1× open access unreviewed
  7. 7
    5-ALA in Oncology: Current Clinical Applications, Biological Limitations, and Emerging Translational Strategies

    Inglot J, Bartusik-Aebisher D, Myśliwiec A, Dynarowicz K, Paul A, Xavierselvan M, Aebisher D. · 2026-06-01

    open access unreviewed
  8. 8
    Homozygous variegate porphyria: Two cases misdiagnosed as erythropoietic protoporphyria.

    Wang C, Bensaber N, Rebeiz L, Tran B, Tortorelli S, Kyle K, Yalom LK, Moghe A, Anderson KE, Leaf RK, Yeung AK. · 2026-06-07

    open access unreviewed
  9. 9
    Novel PPOX Mutation With Ocular Involvement in Variegate Porphyria: Case Report and Literature Review.

    Ramos-Dávila EM, Sayegh Y, Ebehart CG, Thorne JE, Liberman P. · 2026-06-12

    unreviewed
  10. 10
    A Rare Clinical Presentation of Variegate Porphyria.

    Viakhireva I, Murtazina A, Orlova A, Makretskaya N, Nikitin S, Skoblov M. · 2026-06-01

    open access unreviewed

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.