6-Pyruvoyltetrahydropterin synthase
| 6-pyruvoyltetrahydropterin synthase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC number | 4.2.3.12 | ||||||||
| CAS number | 97089-82-2 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / EGO | ||||||||
| |||||||||
In enzymology, a 6-pyruvoyltetrahydropterin synthase (PTPS) (EC 4.2.3.12) is an enzyme that catalyzes the following chemical reaction:
7,8-Dihydroneopterin triphosphate 6-pyruvoyltetrahydropterin + triphosphate
Hence, this enzyme has one substrate, 7,8-Dihydroneopterin triphosphate, and two products, 6-pyruvoyltetrahydropterin and triphosphate.
Enzyme Class
This enzyme belongs to the family of lyases, to be specific, those carbon-oxygen lyases acting on phosphates. The systematic name of this enzyme class is 6-[(1S,2R)-1,2-dihydroxy-3-triphosphooxypropyl]-7,8-dihydropterin triphosphate-lyase (6-pyruvoyl-5,6,7,8-tetrahydropterin-forming).
Synonyms
Other names in common use include 2-amino-4-oxo-6-[(1S,2R)-1,2-dihydroxy-3-triphosphooxypropyl]-7,8-, and dihydroxypteridine triphosphate lyase. This enzyme participates in tetrahydrobiopterin biosynthesis.
Genetics
This enzyme 6-pyruvoyltetrahydropterin synthaseis encoded by the PTS gene.
Clinical significance
6-pyruvoyl-tetrahydropterin synthase deficiency belongs to the rare recessive disorder
Structural studies
As of mid-2010, 13 structures have been solved for this class of enzymes, with PDB accession codes 3M0N, 3LZE, 3LX3, 3I2B, 2DTT, 1GTQ, 1B66, 1B6Z, 1Y13, 2A0S, 2DJ6, 2G64, and 2OBA.
References
- Milstien S, Kaufman S (1989). "The biosynthesis of tetrahydrobiopterin in rat brain. Purification and characterization of 6-pyruvoyl tetrahydropterin (2'-oxo)reductase". J. Biol. Chem. 264 (14): 8066–73. PMID 2656673.
- Thony B, Leimbacher W, Burgisser D, Heizmann CW (1992). "Human 6-pyruvoyltetrahydropterin synthase: cDNA cloning and heterologous expression of the recombinant enzyme". Biochem. Biophys. Res. Commun. 189 (3): 1437–43. doi:10.1016/0006-291X(92)90235-D. PMID 1282802.