NP_058916.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
Molecular Weight
16,241 Da
NCBI Official Full Name
6-pyruvoyl tetrahydrobiopterin synthase
NCBI Official Synonym Full Names
6-pyruvoyl-tetrahydropterin synthase
NCBI Protein Information
6-pyruvoyl tetrahydrobiopterin synthase; PTPS; PTP synthase; 6-pyruvoyltetrahydropterin synthase
UniProt Protein Name
6-pyruvoyl tetrahydrobiopterin synthase
UniProt Synonym Gene Names
UniProt Entry Name
PTPS_RAT
NCBI Summary for PTS
catalyzes the formation of 6-pyruvoyltetrahydrobiopterin in tetrahydrobiopterin biosynthesis [RGD, Feb 2006]
UniProt Comments for PTS
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.
Protein type: Cofactor and Vitamin Metabolism - folate biosynthesis; Mitochondrial; Lyase; EC 4.2.3.12
Cellular Component: mitochondrion; cytoplasm
Molecular Function: identical protein binding; protein homodimerization activity; metal ion binding; 6-pyruvoyltetrahydropterin synthase activity
Biological Process: tetrahydrobiopterin biosynthetic process
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Pathways associated with PTS elisa kit
Diseases associated with PTS elisa kit
Organs/Tissues associated with PTS elisa kit
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