1976
DOI: 10.1002/dev.420090208
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Maternal hyperphenylalaninemia: An experimental model in rats

Abstract: Experimental maternal hyperphenylalaninemia produced in pregnant F344 rats by the combined use of p-Chloro-DL-phenylalanine and L-phenylalanine reduced fetal birth weight in comparison to saline-injected controls. Offspring who experiences hyperphenylalaninemia in utero died within 5 days after birth. Fetal plasma phenylalanine levels were several times higher than maternal plasma phenylalanine levels, indicating that the placenta actively concentrates maternal phenylalanine. Fetal brain phenylalanine levels r… Show more

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Cited by 11 publications
(2 citation statements)
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“…either phe dence of mental retardation and congenital defects in infants due nor PCPA induces long-term effects on water maze to transplacental hyperphenylalaninemia (2-4). Relying upon performance or on any other measure of behavioral ~erformance evidence developed concerning blood brain banier transport of characteristic of PKU animal models using similar treatment amino acids, we sought to reduce the embryotoxic effects of regimens (23,24,(28)(29)(30). On d 1 1 and 15 of gestation, every hypeVhenylalaninemia using the branched-chain amino acids other dam in each group was anesthetized with methoxyflurane in a murine model of PKU.…”
mentioning
confidence: 99%
“…either phe dence of mental retardation and congenital defects in infants due nor PCPA induces long-term effects on water maze to transplacental hyperphenylalaninemia (2-4). Relying upon performance or on any other measure of behavioral ~erformance evidence developed concerning blood brain banier transport of characteristic of PKU animal models using similar treatment amino acids, we sought to reduce the embryotoxic effects of regimens (23,24,(28)(29)(30). On d 1 1 and 15 of gestation, every hypeVhenylalaninemia using the branched-chain amino acids other dam in each group was anesthetized with methoxyflurane in a murine model of PKU.…”
mentioning
confidence: 99%
“…Since dietary treatment of children with classic PKU has resulted in an increased number of PKU females reaching childbearing age, there is a need for more information concerning the developmental processes affected by maternal, as well as classic, PKU.Biochemically, PKU is characterized by a deficit in the normal conversion of phenylalanine to tyrosine, which results in a surplus of phenylalanine and in other related biochemical changes. Earlier animal models of this disease employing phenylalanine in combination with parachloro-phenylalanine produced toxic side effects and death (Anderson, 1976;Copenhaver, Carver, & Schalock, 1974). A recent animal model of maternal PKU utilized increased dietary phenylalanine together with a-methylphenylalanine, which resulted in fewer toxic side effects than phenylalanine with para-chloro-phenylalanine (Brass, Isaacs, McHesney, & Greengard, 1982).…”
mentioning
confidence: 99%