Target intelligence / Profile preview

6-Pyruvoyl Tetrahydrobiopterin Synthase (PTPS)

Target
PTPS
Molecular classification
Enzyme, Lyase
01

Overview

6-Pyruvoyl tetrahydrobiopterin synthase (PTPS) is an enzyme that catalyzes the second step in the de novo biosynthesis of tetrahydrobiopterin (BH4), a critical cofactor for several essential metabolic processes. PTPS converts 7,8-dihydroneopterin triphosphate into 6-pyruvoyltetrahydropterin with the elimination of inorganic triphosphate. Mutations in PTPS can lead to hyperphenylalaninemia due to BH4 deficiency.

02

Mechanism of action

Catalyzes the conversion of 7,8-dihydroneopterin triphosphate to 6-pyruvoyltetrahydropterin

03

Biological functions

Tetrahydrobiopterin biosynthesisAmino acid metabolismNeurotransmitter synthesis
04

Disease associations

HyperphenylalaninemiaBH4-deficient disorders
05

Safety considerations

Potential for off-target effects if directly targeted by drugsDrug interactions related to BH4 metabolism
06

Biomarkers

Dihydroneopterin triphosphate levels6-pyruvoyltetrahydropterin levels

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