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Gene Review

Fah  -  fumarylacetoacetate hydrolase

Rattus norvegicus

Synonyms: Beta-diketonase, FAA, Fumarylacetoacetase, Fumarylacetoacetate hydrolase
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Disease relevance of Fah


High impact information on Fah

  • The number of altered liver cell foci in rats given 200 ppm N-2-fluorenylacetamide (FAA; CAS: 53-96-3) in the diet for 8 weeks and followed by the betel nut diet for 16 weeks was significantly greater than that in animals fed the FAA diet alone [6].
  • Continued ingestion of any of the three hepatocarcinogens, FAA, 3'-methyl-4-dimethylaminoazobenzene, or ethionine, resulted in the progressive loss of the early radioactive complex and the concurrent gain in liver cytosol of the late carcinogen: protein complex (Mr approximately 150,000) formed by the tracer dose of FAA [2].
  • This work examines whether 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione (NTBC), a potent inhibitor of the enzyme 4-hydroxyphenylpyruvate dioxygenase (HPPD) located upstream of FAH, modulates D-serine-induced nephrotoxicity [7].
  • FAH is involved in tyrosine catabolism; hence, this pathway may be involved in mediating the toxicity [7].
  • We describe a new method for the asymmetric synthesis of [(18)F]fluorinated aromatic alpha-amino acids (FAA) under phase transfer conditions using achiral glycine derivative NiPBPGly and (S)-NOBIN as a novel substrate/catalyst pair [8].

Chemical compound and disease context of Fah

  • Tyrosinemia type 1 (HT1) is an autosomal recessive disorder of the tyrosine metabolism in which the fumarylacetoacetate hydrolase enzyme is defective [9].
  • Experiment 2: The incidence of altered hepatocellular foci in female ACI/N rats given N-2-fluorenylacetamide (FAA, 0.02% in diet) for 10 wk and reserpine (weekly subcutaneous injections, 1 microgram/g body weight) during or after (17 wk) FAA exposure was significantly lower than that of rats given FAA alone [10].
  • The toxicity in rats and mice and the responses of 5 rat lung tumours, a rat rhabdomyosarcoma and a mouse colon tumour to flavone acetic acid, FAA, were studied [11].
  • Experiment 3: The number of hepatocellular foci in male ACI/N rats given 0.02% FAA diet for 13 wk and E-5166 by gavage (40 mg/kg body weight, 3 times/wk) for 16 wk after the end of FAA exposure was significantly smaller than that in rats given FAA diet alone [10].

Biological context of Fah

  • The LD50 values of FAA in WAG/Rij and BN rats were about 350 and 525 mg/kg respectively, independent of whether the animals were tumour bearing or not [11].

Anatomical context of Fah

  • In previous studies, administration of a radioactive tracer dose of the liver carcinogen, N-2-fluorenylacetamide (2-acetylaminofluorene; FAA), to normal or carcinogen-fed rats led to the presence of one or two principal labeled carcinogen: protein complexes in liver cytosol [2].
  • As a step towards the cloning of the gene for fumarylacetoacetate hydrolase (FAH), we have purified the FAH mRNA from rat liver by specific immunoadsorption of polysomes [12].

Associations of Fah with chemical compounds

  • FAH was elevated in the plasma of animals treated with 4-AP and D-serine at early time points and returned to baseline levels after 3 weeks [4].
  • One toxicity-associated plasma protein was identified as the cellular enzyme fumarylacetoacetate hydrolase (FAH), which is known to be required for tyrosine metabolism [4].
  • Therefore, FAH may function in a fashion analogous to the aspartate aminotransferase (AST) and alanine aminotransferase (ALT) enzymes that are used to measure liver injury [4].
  • The effects of the anti-rheumatic drugs indometacin, dexamethasone, cyclophosphamide and cyclosporin A (CsA) on IL-6 levels during FAA were determined [13].

Analytical, diagnostic and therapeutic context of Fah


  1. Cloning and expression analysis of a cDNA encoding fumarylacetoacetate hydrolase: post-transcriptional modulation in rat liver and kidney. Labelle, Y., Phaneuf, D., Tanguay, R.M. Gene (1991) [Pubmed]
  2. Late target protein of the carcinogen N-2-fluorenylacetamide in rat liver. Munir, K.M., Blackburn, G.R., Sorof, S. Cancer Res. (1988) [Pubmed]
  3. Attenuation of brain free fatty acid liberation during global ischemia: a model for screening potential therapies for efficacy? Nemoto, E.M., Shiu, G.K., Nemmer, J., Bleyaert, A.L. J. Cereb. Blood Flow Metab. (1982) [Pubmed]
  4. A potential biomarker of kidney damage identified by proteomics: preliminary findings. Bandara, L.R., Kelly, M.D., Lock, E.A., Kennedy, S. Biomarkers (2003) [Pubmed]
  5. Long-term effects of flavone acetic acid on the growth of a rat tumour. Bowler, K., Pearson, J.A. Anticancer Res. (1992) [Pubmed]
  6. Carcinogenicity of betel quid. III. Enhancement of 4-nitroquinoline-1-oxide- and N-2-fluorenylacetamide-induced carcinogenesis in rats by subsequent administration of betel nut. Tanaka, T., Kuniyasu, T., Shima, H., Sugie, S., Mori, H., Takahashi, M., Hirono, I. J. Natl. Cancer Inst. (1986) [Pubmed]
  7. D-serine-induced nephrotoxicity: possible interaction with tyrosine metabolism. Williams, R.E., Lock, E.A. Toxicology (2004) [Pubmed]
  8. Catalytic enantioselective synthesis of 18F-fluorinated alpha-amino acids under phase-transfer conditions using (S)-NOBIN. Krasikova, R.N., Zaitsev, V.V., Ametamey, S.M., Kuznetsova, O.F., Fedorova, O.S., Mosevich, I.K., Belokon, Y.N., Vyskocil, S., Shatik, S.V., Nader, M., Schubiger, P.A. Nucl. Med. Biol. (2004) [Pubmed]
  9. DNA damage and repair in mammalian cells exposed to p-hydroxyphenylpyruvic acid. van Dyk, E., Pretorius, P.J. Biochem. Biophys. Res. Commun. (2005) [Pubmed]
  10. Inhibitory effects of chlorogenic acid, reserpine, polyprenoic acid (E-5166), or coffee on hepatocarcinogenesis in rats and hamsters. Tanaka, T., Nishikawa, A., Shima, H., Sugie, S., Shinoda, T., Yoshimi, N., Iwata, H., Mori, H. Basic Life Sci. (1990) [Pubmed]
  11. Responses of experimental rat tumours and a mouse colon tumour to flavone acetic acid. Kal, H.B., de Graaff, E., Van Berkel, A.H., Goedoen, H.H. In Vivo (1992) [Pubmed]
  12. Purification of mRNA coding for the enzyme deficient in hereditary tyrosinemia, fumarylacetoacetate hydrolase. Nicole, L.M., Valet, J.P., Laberge, C., Tanguay, R.M. Biochem. Cell Biol. (1986) [Pubmed]
  13. Interleukin-6 (IL-6) in adjuvant arthritis of rats and its pharmacological modulation. Theisen-Popp, P., Pape, H., Müller-Peddinghaus, R. Int. J. Immunopharmacol. (1992) [Pubmed]
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