Tyrosine metabolism (WP4506)

This pathway shows the tyrosine degradation pathway as presented in Chapter 2 of the book of Blau (ISBN 3642403360 (978-3642403361)). Disorders resulting from an enzyme defect are highlighted in pink. Red frames mark diagnostically important metabolites.
last edited


L Dupuis, DeSl, Egonw, IreneHemel, GMKeulen, MaintBot, Fehrhart, AgustinGV, Eweitz, and Finterly

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Disease Ontology: tyrosinemia type III tyrosinemia type I tyrosinemia type II inherited metabolic disorder alkaptonuria

Pathway Ontology: tyrosinemia type II pathway tyrosinemia type I pathway alkaptonuria pathway tyrosine degradation pathway tyrosine metabolic pathway tyrosinemia type III pathway tyrosinemia pathway


Label Type Compact Identifier
quinol acetate Metabolite chebi:31128
Thiols Metabolite chebi:29256
Hawkinsin Metabolite pubchem.compound:173909
Homogentisate Metabolite chebi:16169
[CO2] Metabolite chebi:16526
4-hydroxyphenylpyruvate hydroxylase Protein uniprot:A0A0B4J1R4
Succinylacetone Metabolite chebi:87897
4-hydroxyphenylpyruvate dioxygenase Protein uniprot:A0A0B4J1R4
4-Maleylacetoacetate Metabolite chebi:17105
Fumarylacetoacetase Protein uniprot:P16930
Fumarate Metabolite chebi:29806
Acetoacetate Metabolite chebi:13705
Homogentisate 1,2-dioxygenase Protein uniprot:Q93099
4-Hydroxyphenylpyruvate Metabolite chebi:36242
L-tyrosine Metabolite chebi:58315
nitisone Metabolite chebi:50378
Tyrosine aminotransferase Protein uniprot:P17735
4-Hydroxyphenylacetate Metabolite chebi:18101
Succinylacetoacetate Metabolite chebi:87999
4-fumarylacetoacetate(2-) Metabolite hmdb:HMDB0062563
Porphobilinogen Metabolite chebi:17381
p-Hydroxyphenyllactate Metabolite chebi:36659
5-Aminolevulinate Metabolite chebi:17549


  1. Item CB, Mihalek I, Lichtarge O, Jalan A, Vodopiutz J, Muhl A, et al. Manifestation of hawkinsinuria in a patient compound heterozygous for hawkinsinuria and tyrosinemia III. Mol Genet Metab. 2007 Aug;91(4):379–83. PubMed Europe PMC Scholia
  2. Brownlee JM, Heinz B, Bates J, Moran GR. Product analysis and inhibition studies of a causative Asn to Ser variant of 4-hydroxyphenylpyruvate dioxygenase suggest a simple route to the treatment of Hawkinsinuria. Biochemistry. 2010 Aug 24;49(33):7218–26. PubMed Europe PMC Scholia
  3. PubMed Europe PMC Scholia