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EXPERIMENTAL MATERNAL HYPERPHENYLALANINEMTA: DISAGGREGATION OF FETAL BRAIN RIBOSOMES
Author(s) -
Copenhaver J. H.,
Vacanti J. P.,
Carver M. J.
Publication year - 1973
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1973.tb04248.x
Subject(s) - phenylalanine , offspring , polysome , in utero , fetus , amino acid , ribosome , protein biosynthesis , biology , in vitro , biochemistry , endocrinology , medicine , pregnancy , rna , genetics , gene
Disaggregation of polyribosomal structures has been demonstrated in fetal rat brains following treatment of the maternal animal with para ‐chloro‐ d,l ‐phenylalanine (used primarily to inhibit maternal phenylalanine hydroxylase, EC 1.14.3.1) and with phenylalanine (used to raise the level of circulating phenylalanine in maternal and fetal plasma). Since highly disaggregated polyribosomal systems cannot support normal levels of protein synthesis in vitro , it has been postulated that the composition of the free amino acid pool(s) plays a regulatory role in protein synthesis through the intermediary effect of polyribosomal aggregation‐disaggregation. We believe that a possibly prolonged period of disaggregation of neuronal polyribosomes might disrupt neuronal protein synthesis sufficiently in utero to produce the mental insufficiency observed in the offspring of untreated maternal phenylketonurics.