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Protein Page:
PTS (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
PTS 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. Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A); also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet. Belongs to the PTPS family. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; Lyase; Cofactor and Vitamin Metabolism - folate biosynthesis; EC 4.2.3.12
Cellular Component: mitochondrion; cytoplasm; cytosol
Molecular Function: identical protein binding; protein homodimerization activity; metal ion binding; 6-pyruvoyltetrahydropterin synthase activity
Biological Process: amino acid metabolic process; tetrahydrobiopterin biosynthetic process; central nervous system development; regulation of nitric-oxide synthase activity; nitric oxide metabolic process
Reference #:  Q03393 (UniProtKB)
Alt. Names/Synonyms: 6-pyruvoyl tetrahydrobiopterin synthase; 6-pyruvoyltetrahydropterin synthase; FLJ97081; PTP synthase; PTPS; PTS
Gene Symbols: PTS
Molecular weight: 16,386 Da
Basal Isoelectric point: 6.2  Predict pI for various phosphorylation states
Select Structure to View Below

PTS

Protein Structure Not Found.


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Sites Implicated In
enzymatic activity, induced: S19‑p

Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
1 9 S19-p AQVSRRIsFSASHRL
0 5 S28 SASHRLYSkFLSDEE
0 2 K29-ub ASHRLYSkFLSDEEN
0 2 F30 SHRLYSkFLSDEENL
0 1 K38-ub LSDEENLkLFGKCNN
0 44 Y128-p VLPVGVLyKVKVYET
0 1 K143-ub DNNIVVYkGE_____
  mouse

 
S18-p ARLSRLVsFSASHRL
S27-p SASHRLHsPsLSDEE
P28 ASHRLHsPsLSDEEN
S29-p SHRLHsPsLSDEENL
R37 LSDEENLRVFGKCNN
Y127 LLPVGALYKVKVFET
K142 DNNIVVYKGE_____
  rat

 
S18 ARLSRLVSFSASHRL
S27-p SASHRLHsPSLSAEE
P28 ASHRLHsPSLSAEEN
S29 SHRLHsPSLSAEENL
K37 LSAEENLKVFGKCNN
Y127 LLPVGALYKVKVYET
K142 DNNIVVYKGE_____
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