L-phenylalanine degradation IV (mammalian, via side chain)

Williams RA, Mamotte CD, Burnett JR. Phenylketonuria: an inborn error of phenylalanine metabolism. Clin Biochem Rev. 2008 Feb;29(1):31–41. PMID: 18566668; PMCID: PMC2423317.; Wada M. Measurement of hepatic phenylalanine metabolism in children using the [(13)C]-phenylalanine breath test and gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2004 Jun 25;806(1):5–10. doi: 10.1016/j.jchromb.2004.02.023. PMID: 15149604.; Bier DM. Amino acid pharmacokinetics and safety assessment. J Nutr. 2003 Jun;133(6 Suppl 1):2034S–2039S. doi: 10.1093/jn/133.6.2034s. PMID: 12771361.; Kaufman S. A model of human phenylalanine metabolism in normal subjects and in phenylketonuric patients. Proc. Natl. Acad. Sci. U.S.A. 1999 Mar 16;96(6):3160–4. doi: 10.1073/pnas.96.6.3160.; Lindblad B, Lindstedt G, Lindstedt S, Rundgren M. Purification and some properties of human 4-hydroxyphenylpyruvate dioxygenase (I). Journal of Biological Chemistry. 1977 Jul;252(14):5073–84. doi: 10.1016/s0021-9258(17)40160-8.; Yang H-T, Neff NH. ß-PHENYLETHYLAMINE: A SPECIFIC SUBSTRATE FOR TYPE B MONOAMINE OXIDASE OF BRAIN. The Journal of Pharmacology and Experimental Therapeutics. 1973 Nov;187(2):365–71. doi: 10.1016/s0022-3565(25)29682-3.; Fellman JH, Fujita TS, Roth ES. Assay, properties and tissue distribution of p-hydroxyphenylpyruvate hydroxylase. Biochimica et Biophysica Acta (BBA) - Enzymology. 1972 Sep;284(1):90–100. doi: 10.1016/0005-2744(72)90048-4.

Metabolites

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Diphosphate

Formula: H4P2O7 (177.9432294)

CAS ID: 2466-09-3

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Hydrogen peroxide

Formula: H2O2 (34.0054792)

CAS ID: 7722-84-1

Lithium

Formula: Li (7.016005)

CAS ID: 7439-93-2

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

Nickel(2+)

Formula: Ni (57.935347)

CAS ID: 14701-22-5

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Phenylacetaldehyde

Formula: C8H8O (120.0575118)

CAS ID: 122-78-1

Potassium cation

Formula: K (38.963708)

CAS ID: 24203-36-9

Rubidium cation

Formula: Rb (84.9118)

CAS ID: 22537-38-8

Sodium cation

Formula: Na (22.98977)

CAS ID: 17341-25-2

Zinc cation

Formula: Zn (63.929145)

CAS ID: 23713-49-7

2-oxo-3-phenylpropanoate

Formula: C9H7O3 (163.0395172)

CAS ID: 620-76-8

2-oxoglutarate(2-)

Formula: C5H4O5 (144.00587339999998)

CAS ID: 64-15-3

phenylacetate

Formula: C8H7O2 (135.0446022)

CAS ID:

(2-hydroxyphenyl)acetate

Formula: C8H7O3 (151.0395172)

CAS ID:

benzoyl-CoA(4-)

Formula: C28H36N7O17P3S (867.1101196000001)

CAS ID:

adenosine 5'-monophosphate(2-)

Formula: C10H12N5O7P (345.0474332)

CAS ID:

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

NADH(2-)

Formula: C21H27N7O14P2 (663.1091182000001)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9

Dithionitrobenzoic acid

Formula: C14H6N2O8S2-2 (393.9565596)

CAS ID:



Enzyme

EC Number name full name note
1.13.11.27 4-hydroxyphenylpyruvate dioxygenase 4-hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating, decarboxylating)
1.2.1.3 aldehyde dehydrogenase (NAD+) aldehyde:NAD+ oxidoreductase
1.2.1.94 farnesal dehydrogenase farnesal:NAD+ oxidoreductase
1.4.3.21 primary-amine oxidase primary-amine:oxygen oxidoreductase (deaminating)
1.4.3.4 monoamine oxidase amine:oxygen oxidoreductase (deaminating)
2.6.1.1 aspartate transaminase L-aspartate:2-oxoglutarate aminotransferase
2.6.1.7 kynurenine-oxoglutarate transaminase L-kynurenine:2-oxoglutarate aminotransferase
4.1.1.28 aromatic-L-amino-acid decarboxylase Aromatic-L-amino-acid carboxy-lyase


Proteins

Protein ID name full name