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MS
unreviewed

Fumarylacetoacetate hydrolase (FAH)

1 reactions · 1 pathways

Cofactors used

Clinical / pharmacological. Tyrosinaemia type I: liver failure, renal Fanconi, cabbage odour; treated with nitisinone

What it does, reaction by reaction

1 reactions

Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion

step 14 Irreversible/directional

Fumarylacetoacetate + H₂O → fumarate + acetoacetate

Converts Fumarylacetoacetate H2O into Fumarate Acetoacetate

› Notes

Fumarylacetoacetate hydrolase (FAH) yields one glucogenic product (fumarate) and one ketogenic product (acetoacetate). This establishes phenylalanine and tyrosine as both glucogenic and ketogenic.

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

Europe PMC · fetched just now · sorted by publication date

  1. 1
    Transient culture of adult human hepatocytes as organoids improves engineered liver engraftment.

    Saxton SH, Kang I, Mitchell S, Lotto J, Upadhyay A, Zou C, Quirk C, McCray TN, Prado O, Johansson F, Fortin C… · 2026-07-23

    open access unreviewed
  2. 2
    Man With Blurry Vision.

    Wu PT, Chong CF. · 2026-09-23

    open access unreviewed
  3. 3
  4. 4
    Effects of cassava wax immersion on pain, pressure pain threshold, and upper extremity function among patients with trigger finger.

    Khamnon N, Buttagat V, Punyanitya S, Kaewjoho C, Kluayhomthong S, Areeudomwong P. · 2026-05-26

    open access unreviewed
  5. 5
    Cross-Sectional coaxial technique: optimizing specimen adequacy and safety in ultrasound-guided renal biopsy procedures.

    Huang L, Zhan X, Chen L, Zhang J, Luo N, Wang Y, Wang D, Liu J, Ren L, Li M, Yang Q, Luo H. · 2026-08-18

    open access unreviewed
  6. 6
    Ginger-based formulations for allergic rhinitis disease: a systematic review and meta-analysis of experimental studies in animals and humans.

    Inpan R, Kantasa T, Hanprasertpong N, Na Takuathung M, Koonrungsesomboon N. · 2026-05-25

    open access unreviewed
  7. 7
    Growth, Carcass Quality and Gut Microbiota Responses to Extruded Rapeseed Meal With Protease in Broiler Chickens.

    Hameed R, Tahir M, Khan RU, Ahmad S, Rossi L, Al-Akeel RK. · 2026-11-01

    open access unreviewed
  8. 8
  9. 9
    Author Correction: Surveillance of avian influenza through bird guano in remote regions of the global south to uncover transmission dynamics.

    Wannigama DL, Amarasiri M, Phattharapornjaroen P, Hurst C, Modchang C, Besa JJV, Miyanaga K, Cui L, Fernandez… · 2026-10-01

    open access unreviewed
  10. 10
    A noncanonical function of the tyrosine degradation enzyme FAH drives CDK4/6 inhibitor resistance in breast cancer.

    Högström J, Doh HM, Kump A, Petrova B, Wang P, Lee JD, Koivula K, Kozlova N, Cruz KA, Hong SM, Perea C, Garg … · 2026-09-30

    open access unreviewed

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