Tyrosine Metabolism (Homo sapiens)

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1333Tyrosinaemia Type IIITyrosinaemia Type IHawkinsinuria (HAWK)Tyrosinaemia Type IHawkinsinThiolsquinol acetate32Homogentisate[CO2]4-hydroxyphenylpyruvate hydroxylaseSuccinylacetone24-hydroxyphenylpyruvate dioxygenase4-MaleylacetoacetateFumarylacetoacetaseFumarateAcetoacetateHomogentisate 1,2-dioxygenase4-HydroxyphenylpyruvateTyrosinenitisoneTyrosine aminotransferase4-HydroxyphenylacetateSuccinylacetoacetate4-fumarylacetoacetate(2-)Porphobilinogenp-Hydroxyphenyllactate5-AminolevulinateAlkaptonuria


This pathway shows the tyrosine degradation pathway as presented in Chapter 2 of the book of Blau (ISBN 978-3-642-40337-8). Disorders resulting from an enzyme defect are highlighted in pink. Red frames mark diagnostically important metabolites.

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Ontology Terms



  1. Item CB, Mihalek I, Lichtarge O, Jalan A, Vodopiutz J, Muhl A, Bodamer OA; ''Manifestation of hawkinsinuria in a patient compound heterozygous for hawkinsinuria and tyrosinemia III.''; Mol Genet Metab, 2007 PubMed Europe PMC
  2. Blau, Nenad, Duran, Marinus, Gibson, K. Michael, Dionisi-Vici, Carlo; ''Physician's Guidee to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; , 2014
  3. 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 PubMed Europe PMC


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106201view12:09, 16 August 2019MaintBotHMDB identifier normalization
104393view13:59, 23 May 2019GMKeulenAdded Rhea identifiers and CheBi identifiers
104019view17:35, 25 April 2019IreneHemelModified description
102787view13:24, 24 January 2019EgonwReplaced secondary ChEBI IDs with primary IDs.
102783view10:23, 24 January 2019DeSlOntology Term : 'alkaptonuria pathway' added !
102782view10:23, 24 January 2019DeSlOntology Term : 'alkaptonuria' added !
102781view10:22, 24 January 2019DeSlCreated unique ref for Pubmed 20677779
102780view10:18, 24 January 2019DeSlAdded lit. ref for reaction to Hawkinsin.
102779view10:04, 24 January 2019DeSlAdded side reaction to Hawkinsin.
102777view09:25, 24 January 2019DeSlChanged layout, added last disease, converted lines to disease node to graphical lines.
102759view12:50, 23 January 2019L DupuisModified description
102758view12:47, 23 January 2019L DupuisFramed diagnostically important metabolites
102757view12:42, 23 January 2019L DupuisAdded reference
102756view12:36, 23 January 2019L DupuisLinked omim ids to the disorders
102755view12:24, 23 January 2019L DupuisConverted interaction lines to graphical interface lines to indicate a point where pathology could occur
102754view11:56, 23 January 2019L DupuisConnected unconnected lines
102753view10:06, 23 January 2019L DupuisOntology Term : 'inherited metabolic disorder' added !
102752view10:04, 23 January 2019L DupuisOntology Term : 'tyrosine metabolic pathway' added !
102751view10:03, 23 January 2019L DupuisOntology Term : 'tyrosine degradation pathway' added !
102750view09:58, 23 January 2019L DupuisNew pathway

External references


View all...
NameTypeDatabase referenceComment
4-HydroxyphenylacetateMetaboliteCHEBI:18101 (ChEBI)
4-HydroxyphenylpyruvateMetaboliteCHEBI:36242 (ChEBI)
4-MaleylacetoacetateMetaboliteCHEBI:17105 (ChEBI)
4-fumarylacetoacetate(2-)MetaboliteHMDB62563 (HMDB)
4-hydroxyphenylpyruvate dioxygenaseProteinA0A0B4J1R4 (Uniprot-TrEMBL) HPD gene, without mutation
4-hydroxyphenylpyruvate hydroxylaseProteinA0A0B4J1R4 (Uniprot-TrEMBL) HPD gene, with mutation p.Asn241Ser, leading to a change in function in the protein.
5-AminolevulinateMetaboliteCHEBI:17549 (ChEBI)
AcetoacetateMetaboliteCHEBI:15344 (ChEBI)
FumarateMetaboliteCHEBI:18012 (ChEBI)
FumarylacetoacetaseProteinP16930 (Uniprot-TrEMBL)
HawkinsinMetabolite173909 (PubChem-compound) aka 2-cystenyl-1,4-dihydroxycyclohexenylacetate
Homogentisate 1,2-dioxygenaseProteinQ93099 (Uniprot-TrEMBL)
HomogentisateMetaboliteCHEBI:16169 (ChEBI)
PorphobilinogenMetaboliteCHEBI:17381 (ChEBI)
SuccinylacetoacetateMetaboliteCHEBI:87999 (ChEBI)
SuccinylacetoneMetaboliteCHEBI:87897 (ChEBI)
ThiolsMetaboliteCHEBI:29256 (ChEBI)
Tyrosine aminotransferaseProteinP17735 (Uniprot-TrEMBL)
TyrosineMetaboliteCHEBI:18186 (ChEBI)
[CO2]MetaboliteCHEBI:16526 (ChEBI)
nitisoneMetaboliteCHEBI:50378 (ChEBI) AKA NTBC
p-HydroxyphenyllactateMetaboliteCHEBI:36659 (ChEBI)
quinol acetateMetaboliteCHEBI:31128 (ChEBI) This compound is annotated with an example for quinol acetate (4-hydroxyphenyl acetate).

Annotated Interactions

No annotated interactions

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