Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

4.2.3.12

Official Name

6-pyruvoyltetrahydropterin synthase

Name from literature

pyruvoyl tetrahydropterin synthase

Pathway from literature

synthesizing BH4/the synthesis of human tetrahydrobiopterin

Pathway from KEGG

Metabolism of Cofactors and Vitamins; Folate biosynthesis; map00790

Organisms

Rat (10116)

Genome localization

8q23[29498 ],

Comments

Catalyses triphosphate elimination and an intramolecular redox reaction in the presence of Mg2+. It has been identified in human liver. This enzyme is involved in the de novo synthesis of tetrahydrobiopterin from GTP, with the other enzymes involved being EC 1.1.1.153 (sepiapterin reductase) and EC 3.5.4.16 (GTP cyclohydrolase I) [3].

Rate-limiting Description

"6-Pyruvoyl-tetrahydropterin synthase is the rate-limiting enzyme in the synthesis of human tetrahydrobiopterin, a cofactor for several hydroxylases involved in catecholamine and serotonin biosynthesis." (8307017)

Regulatory Information

Regulatory type

Detail

phosphorylation;

P27213:from_uniprot:18_Phosphoserine

Gene ontology

Gene ontology

GO:0008270 (F) zinc ion binding [P27213 ];
GO:0006729 (P) tetrahydrobiopterin biosynthetic process [P27213 ];
GO:0006559 (P) L-phenylalanine catabolic process [P27213 ];
GO:0003874 (F) 6-pyruvoyltetrahydropterin synthase activity [P27213 ];

Disease relevance

Disease

Deficiency leads to phenylketonuria [P27213 ];

Links

SwissProt

P27213

Entrez Gene

29498



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  Last Modified: 2009-03-24  
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