2636-52-4

7-Biopterin is a 7-substituted pterin. It is formed by non-enzymatic rearrangement of 4a-hydroxy-tetrahydropterin in the absence of pterin-4a-carbinolamine dehydratase (PCD) in vitro and levels are elevated in the urine of hyperphenylalaninemia patients carrying heterozygous mutations in the PCBD gene encoding PCD.

7-Substituted pterins, a new class of mammalian pteridines. Primapterin, Anapterin, and 6-Oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia.