title: Phenylalanine hydroxylase activity in preterm infants: is tyrosine a conditionally essential amino acid?
abstract: To assess the production of the nonessential amino acid tyrosine in preterm infants, we estimated the activity of phenylalanine hydroxylase (PAH) in three groups of infants by measuring the conversion of phenylalanine to tyrosine, using a model based on a primed constant 200-min intravenous infusion of [2H5]phenylalanine. We determined the isotopic enrichments of [2H5]phenylalanine and [2H4]tyrosine by selected-ion-monitoring gas chromatography-mass spectrometry (GCMS). Group 1 (n = 7, mean gestational age 29.7 +/- 1.5 wk, mean birth weight 1.4 +/- 0.4 kg) was studied during the first 4 d of life before initiation of amino acid nutrition. Group 2 (n = 7, mean gestational age 29.7 +/- 1.5 wk, mean birth weight 1.4 +/- 0.4 kg) was studied at 4-6 d of life after receiving amino acid nutrition. Group 3 (n = 4, mean gestational age 28.5 +/- 0.9 wk, mean birth weight 1.1 +/- 0.1 kg) was studied during the first 4 d of life after receiving amino acid nutrition. Calculated from the observed enrichments, phenylalanine conversion to tyrosine was 5.9 +/- 2.6, 19.4 +/- 8.8 and 11 +/- 1.8 mumol.kg-1l.h-1 in groups 1, 2, and 3, respectively. The rate of conversion of phenylalanine to tyrosine increased significantly after initiation of amino acid nutrition. We conclude that preterm infants are capable of converting phenylalanine to tyrosine. Provision of phenylalanine in the context of parenteral amino acid nutrition solution accelerated PAH conversion of phenylalanine to tyrosine, suggesting that the enzyme system is capable of responding normally to provision of substrate.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis Children's Hospital, Missouri, USA.
authors.forename: R A
authors.initials: RA
authors.lastname: Kilani
authors.forename: F S
authors.initials: FS
authors.lastname: Cole
authors.forename: D M
authors.initials: DM
authors.lastname: Bier
mesh_terms: D001724:Birth Weight; D002149:Energy Intake; D008401:Gas Chromatography-Mass Spectrometry; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature / Q000378:metabolism*; D007262:Infusions, Intravenous; D010649:Phenylalanine / Q000008:administration & dosage / Q000378:metabolism*; D010651:Phenylalanine Hydroxylase / Q000378:metabolism*; D014443:Tyrosine / Q000378:metabolism*
chemical_list: D014443:Tyrosine; D010649:Phenylalanine; D010651:Phenylalanine Hydroxylase