PTS

PTS catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity.

Full Name

6-pyruvoyltetrahydropterin synthase

Function

Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin.

Biological Process

Cellular amino acid metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Central nervous system developmentManual Assertion Based On ExperimentTAS:ProtInc
Tetrahydrobiopterin biosynthetic processManual Assertion Based On ExperimentTAS:ProtInc

Cellular Location

cytoplasm
cytosol
mitochondrion

Involvement in disease

Hyperphenylalaninemia, BH4-deficient, A (HPABH4A):
An autosomal recessive disorder characterized by hyperphenylalaninemia, depletion of the neurotransmitters dopamine and serotonin, and progressive cognitive and motor deficits. Neurological symptoms are unresponsive to the classic phenylalanine-low diet.

PTM

Phosphorylation of Ser-19 is required for maximal enzyme activity.