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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.

[1] B TH?NY. 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).[J]. American journal of human genetics, 1998, 62 6: 1302-1311. DOI: 10.1086/301887
[2] H.-CH. CURTIUS . Primapterin, anapterin, and 6-oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia[J]. Biochemical and biophysical research communications, 1988, 153 2: Pages 715-721. DOI: 10.1016/s0006-291x(88)81153-7