Primapterin, anapterin, and 6-oxo-primapterin, three new 7-substituted pterins identified in a patient with hyperphenylalaninemia.
Three unknown compounds present in the urine of a patient with mild hyperphenylalaninemia were identified to be L-erythro-7-iso-biopterin, D-erythro-7-iso-neopterin, and L-erythro-6-oxo-7-iso-biopterin. The newly identified pterins were named primapterin, anapterin, and 6-oxo-primapterin, respectively. Primapterin and anapterin are present in very low concentrations in every human urine, as well as in the liver of man and mouse, whereas 6-oxo-primapterin was detected in the patient's urine only. Substantial amounts of primapterin were excreted in the patient described. The metabolic origin of primapterin and anapterin is still obscure.
Curtius
H C
HC
Department of Pediatrics, University of Zurich, Switzerland.
Kuster
T
T
Matasovic
A
A
Blau
N
N
Dhondt
J L
JL
D000818:Animals; D001708:Biopterins / Q000031:analogs & derivatives* / Q000652:urine; D006801:Humans; D013058:Mass Spectrometry; D051379:Mice; D010649:Phenylalanine / Q000097:blood*
C056247:6-oxoprimapterin; C056248:anapterin; D001708:Biopterins; C056246:primapterin; D010649:Phenylalanine