UniProt Accession

 PTPS_HUMAN; Q03393; B0YJ87; Q8WVG8 

Genbank Protein ID

 M97655; D17400; D25234; L76259; U63383; U63380; U63381; U63382; AB042297; EF445018; CH471065; BC009686; BC018029 

Genbank Nucleotide ID

 AAA51541.1; BAA04224.1; BAA04959.1; AAB64229.1; AAC16970.1; AAC16970.1; AAC16970.1; AAC16970.1; BAA95486.1; ACA06065.1; EAW67195.1; AAH09686.1; AAH18029.1 

Protein Name

 6-pyruvoyl tetrahydrobiopterin synthase 

Protein Synonyms/Alias

 PTP synthase; PTPS 

Gene Name

 PTS 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
29	ASHRLYSKFLSDEEN	ubiquitination	[1, 2, 3, 4]
38	LSDEENLKLFGKCNN	ubiquitination	[1, 2, 3, 4, 5, 6]
143	DNNIVVYKGE*****	ubiquitination	[2, 3, 6]

Reference

 [1] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [2] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [3] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]
 [4] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789]
 [5] Systems-wide analysis of ubiquitylation dynamics reveals a key role for PAF15 ubiquitylation in DNA-damage bypass.
 Povlsen LK, Beli P, Wagner SA, Poulsen SL, Sylvestersen KB, Poulsen JW, Nielsen ML, Bekker-Jensen S, Mailand N, Choudhary C.
 Nat Cell Biol. 2012 Oct;14(10):1089-98. [PMID: 23000965]
 [6] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 

Functional Description

 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. 

Sequence Annotation

 ACT_SITE 43 43 Proton acceptor (By similarity).
 ACT_SITE 90 90 Charge relay system (By similarity).
 ACT_SITE 134 134 Charge relay system (By similarity).
 METAL 24 24 Zinc (By similarity).
 METAL 49 49 Zinc (By similarity).
 METAL 51 51 Zinc (By similarity).
 MOD_RES 19 19 Phosphoserine; by PKG.  

Keyword

 3D-structure; Complete proteome; Disease mutation; Lyase; Metal-binding; Phenylketonuria; Phosphoprotein; Reference proteome; Tetrahydrobiopterin biosynthesis; Zinc. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 145 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; TAS:Reactome.
 GO:0005739; C:mitochondrion; IDA:LIFEdb.
 GO:0003874; F:6-pyruvoyltetrahydropterin synthase activity; TAS:Reactome.
 GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
 GO:0006520; P:cellular amino acid metabolic process; TAS:ProtInc.
 GO:0007417; P:central nervous system development; TAS:ProtInc.
 GO:0046209; P:nitric oxide metabolic process; TAS:Reactome.
 GO:0050999; P:regulation of nitric-oxide synthase activity; TAS:Reactome.
 GO:0006729; P:tetrahydrobiopterin biosynthetic process; TAS:ProtInc. 

Interpro

 IPR007115; 6-PTP_synth/QueD.
 IPR022470; PTPS_Cys_AS.
 IPR022469; PTPS_His_AS. 

Pfam

 PF01242; PTPS 

SMART

  

PROSITE

 PS00987; PTPS_1
 PS00988; PTPS_2 

PRINTS