Tyrosine metabolism and related disorders

Hendrikse NM, Holmberg Larsson A, Svensson Gelius S, Kuprin S, Nordling E, Syrén P. Exploring the therapeutic potential of modern and ancestral phenylalanine/tyrosine ammonia-lyases as supplementary treatment of hereditary tyrosinemia. Scientific Reports. 2020 Jan 28;10(1):1315. doi: 10.1038/s41598-020-57913-y.

Metabolites

4-Coumarate

Formula: C9H8O3 (164.0473418)

CAS ID: 7400-08-0

4-Fumarylacetoacetate

Formula: C8H8O6 (200.0320868)

CAS ID: 28613-33-4

4-Hydroxyphenyl acetate

Formula: C8H8O3 (152.0473418)

CAS ID: 3233-32-7

4-Hydroxyphenylacetate

Formula: C8H8O3 (152.0473418)

CAS ID: 156-38-7

5-Aminolevulinate

Formula: C5H9NO3 (131.0582404)

CAS ID: 106-60-5

Ammonia

Formula: NH3 (17.0265478)

CAS ID: 7664-41-7

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Hydrogen sulfide

Formula: H2S (33.9877212)

CAS ID: 7783-06-4

L-Phenylalanine

Formula: C9H11NO2 (165.0789746)

CAS ID: 63-91-2

Porphobilinogen

Formula: C10H14N2O4 (226.0953524)

CAS ID: 487-90-1

trans-Cinnamate

Formula: C9H8O2 (148.0524268)

CAS ID: 140-10-3

Succinylacetone

Formula: C7H10O4 (158.057906)

CAS ID: 51568-18-4

Hawkinsin

Formula: C11H17NO6S (291.07765420000004)

CAS ID: 63224-90-8

Nitisinone

Formula: C14H10F3NO5 (329.0511046)

CAS ID: 104206-65-7

Succinylacetoacetate

Formula: C8H10O6 (202.04773600000001)

CAS ID: 65115-74-4

acetoacetate

Formula: C4H5O3 (101.02386800000001)

CAS ID: 141-81-1

butenedioate

Formula: C4H2O4 (113.99530920000001)

CAS ID: 17013-01-3

homogentisate

Formula: C8H7O4 (167.0344322)

CAS ID:

3-(4-hydroxyphenyl)pyruvate

Formula: C9H7O4 (179.0344322)

CAS ID: 622-54-8



Enzyme

EC Number name full name note
1.13.11.27 4-hydroxyphenylpyruvate dioxygenase 4-hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating, decarboxylating)
1.13.11.5 homogentisate 1,2-dioxygenase homogentisate:oxygen 1,2-oxidoreductase (decyclizing)
2.6.1.5 tyrosine transaminase L-tyrosine:2-oxoglutarate aminotransferase
3.7.1.2 fumarylacetoacetase 4-fumarylacetoacetate fumarylhydrolase


Proteins

Protein ID name full name