4.2.1.96	(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin	-	
4.2.1.96	additional information	enzyme plays a role in the regulation of expression of phenylalanine hydroxylase via transcription factor HNF1alpha	
4.2.1.96	additional information	-	
4.2.1.96	additional information	a defect in 4a-hydroxytetrahydrobiopterin dehydratase provoces the conversion of the 6-substituted pterins to their 7-substituted isomers	
4.2.1.96	additional information	the enzyme may not play an important role in the regulation of the synthesis of those neurotransmitters which are derived from the hydroxylated aromatic amino acids	
4.2.1.96	additional information	the enzyme is essential in vivo to prevent rearrangement of 4a-hydroxy-6(R)-tetrahydrobiopterin and to maintain the supply of tetrahydrobiopterin cofactor for hydroxylases under conditions where the nonenzymatic rate would be inadequate	
4.2.1.96	additional information	the mutant enzyme form C82R and the 18-amino acid-truncated mutant Glu87-termination occur naturally associated with hyperphenyalaninemia	
4.2.1.96	additional information	the codevelopment of 4a-hydroxytetrahydropterin dehydratase with dihydropteridine reductase strongly supports a physiologically significant role for the dehydratase in tetrahydrobiopterin regeneration	
4.2.1.96	additional information	the cytosolic enzyme is involved in the regeneration of tetrahydrobiopterin, the cofactor of aromatic amino acid monooxygenases	
4.2.1.96	additional information	the enzyme is essentially identical to a cofactor that regulates dimerization of a nuclear homeodomain-containing protein involved in transcription	
4.2.1.96	additional information	both PhhB and phenylalanine hydroxylase (PhhA) are induced coordinately in the presence of either L-tyrosine or L-phenylalanine, but PhhB exhibits a significant basal level of activity that is lacking for PhhA. PhhA and PhhB form a protein-protein complex	
4.2.1.96	additional information	dehydratase/DCoHalpha, has the kinetic properties necessary for regenerating tetrahydrobiopterin cofactor for phenylalanine hydroxylase. Properties of dehydratase/DCoHalpha are consistent with the hypothesis that the activity of this isozyme could account for the relatively mild symptoms reported for patients with a defect in dehydratase/DCoH