7-Biopterin

Description: 7-Biopterin is a 7-substituted pterin.1It is formed by non-enzymatic rearrangement of 4a-hydroxy-tetrahydropterin in the absence of pterin-4a-carbinolamine dehydratase (PCD)in vitroand levels are elevated in the urine of hyperphenylalaninemia patients carrying heterozygous mutations in the PCBD gene encoding PCD.1,2. Target: Others. Smiles: O=C1C=2C(NC([C@H]([C@H](C)O)O)=CN2)=NC(N)=N1. References: Th?ny, B., Neuheiser, F., Kierat, L., et al.Hyperphenylalaninemia with high levels of 7-biopterin is associated with mutations in the PCBD gene encoding the bifunctional protein pterin-4a-carbinolamine dehydratase and transcriptional coactivator (DCoH)Am. J. Hum. Genet.62(6)1302-1311(1998)