title: Effects of dietary mixtures of amino acids on fetal growth and maternal and fetal amino acid pools in experimental maternal phenylketonuria.
abstract: BACKGROUND
  Branched-chain amino acids have been reported to improve fetal brain development in a rat model in which maternal phenylketonuria (PKU) is induced by the inclusion of an inhibitor of phenylalanine hydroxylase, DL-p-chlorophenylalanine, and L-phenylalanine in the diet.
  
  
  OBJECTIVE
  We studied whether a dietary mixture of several large neutral amino acids (LNAAs) would improve fetal brain growth and normalize the fetal brain amino acid profile in a rat model of maternal PKU induced by DL-alpha-methylphenylalanine (AMPhe).
  
  
  METHODS
  Long-Evans rats were fed a basal diet or a similar diet containing 0.5% AMPhe + 3.0% L-phenylalanine (AMPhe + Phe diet) from day 11 until day 20 of gestation in experiments to test various mixtures of LNAAs. Maternal weight gains and food intakes to day 20, fetal body and brain weights at day 20, and fetal brain and fetal and maternal plasma amino acid concentrations at day 20 were measured.
  
  
  RESULTS
  Concentrations of phenylalanine and tyrosine in fetal brain and in maternal and fetal plasma were higher and fetal brain weights were lower in rats fed the AMPhe + Phe diet than in rats fed the basal diet. However, fetal brain growth was higher and concentrations of phenylalanine and tyrosine in fetal brain and in maternal and fetal plasma were lower in rats fed the AMPhe + Phe diet plus LNAAs than in rats fed the diet containing AMPhe + Phe alone.
  
  
  CONCLUSIONS
  LNAA supplementation of the diet improved fetal amino acid profiles and alleviated most, but not all, of the depression in fetal brain growth observed in this model of maternal PKU.
authors: Department of Animal Science, Cornell University, Ithaca, NY 14853, USA. bjs12@cornell.edu
authors: R E
authors: RE
authors: Austic
authors: C L
authors: CL
authors: Su
authors: B J
authors: BJ
authors: Strupp
authors: D A
authors: DA
authors: Levitsky
mesh_terms: D000597:Amino Acids, Branched-Chain / Q000008:administration & dosage / Q000097:blood / Q000494:pharmacology*; D000818:Animals; D001835:Body Weight / Q000187:drug effects; D001921:Brain / Q000196:embryology* / Q000254:growth & development; D001923:Brain Chemistry; D004032:Diet; D005314:Embryonic and Fetal Development / Q000187:drug effects; D005260:Female; D008431:Maternal-Fetal Exchange; D010649:Phenylalanine / Q000031:analogs & derivatives; D010661:Phenylketonurias / Q000139:chemically induced / Q000378:metabolism*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000139:chemically induced / Q000378:metabolism*; D051381:Rats; D020318:Rats, Long-Evans
chemical_list: D000597:Amino Acids, Branched-Chain; C012436:alpha-methylphenylalanine; D010649:Phenylalanine