Names & Taxonomy

Uniprot ID:
Q03393
Entry Name:
PTPS_HUMAN
Status:
reviewed
Protein Names:
6-pyruvoyl tetrahydrobiopterin synthase (PTP synthase) (PTPS) (EC 4.2.3.12)
Gene Names:
PTS
Gene Names Primary:
PTS
Organism:
Homo sapiens (Human)

Structure

Length:
145
Sequence:
MSTEGGGRRCQAQVSRRISFSASHRLYSKFLSDEENLKLFGKCNNPNGHGHNYKVVVTVHGEIDPATGMVMNLADLKKYMEEAIMQPLDHKNLDMDVPYFADVVSTTENVAVYIWDNLQKVLPVGVLYKVKVYETDNNIVVYKGE
Proteomes:
UP000005640

Function

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.
Pathway:
Cofactor biosynthesis; tetrahydrobiopterin biosynthesis; tetrahydrobiopterin from 7,8-dihydroneopterin triphosphate: step 1/3.
Catalytic Activity:
7,8-dihydroneopterin 3'-triphosphate = 6-pyruvoyl-5,6,7,8-tetrahydropterin + triphosphate.
Cofactor:
COFACTOR: Name=Zn(2+); Xref=ChEBI:CHEBI:29105;
Kinetics:
BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=8.1 uM for 7,8-dihydroneopterin triphosphate
Active Site:
ACT_SITE 43 43 Proton acceptor.
Gene Ontology Go:
cytoplasm
cytosol
mitochondrion
6-pyruvoyltetrahydropterin synthase activity
identical protein binding
metal ion binding
cellular amino acid metabolic process
central nervous system development
nitric oxide metabolic process
regulation of nitric-oxide synthase activity
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
Gene Ontology Biological Process:
cellular amino acid metabolic process
central nervous system development
nitric oxide metabolic process
regulation of nitric-oxide synthase activity
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
Gene Ontology Molecular Function:
6-pyruvoyltetrahydropterin synthase activity
identical protein binding
metal ion binding
Gene Ontology Cellular Component:
cytoplasm
cytosol
mitochondrion
Keywords:
3D-structure
Complete proteome
Disease mutation
Lyase
Metal-binding
Phenylketonuria
Phosphoprotein
Reference proteome
Tetrahydrobiopterin biosynthesis
Zinc
Interacts With:
Itself; P38432; Q8TBB1; O00560; Q9P2Z0

Publication

PubMed ID:
1282802 8216273 7698774 8841415 15489334 18669648 20068231 24275569 10531334 9222755 8178819 7493990 8707300 9222757 9159737 9450907 10585341 10220141 10874306 11388593