Literature DB >> 245312

Experimentally induced and natural recovery from the effects of phenylalanine on brain protein synthesis.

J V Hughes, T C Johnson.   

Abstract

The decrease in the neural polyribosomes produced during hyperphenylalaninemia could not be restored to normal levels by the injection of other single neutral amino acids. All of the neutral amino acids that are transported with phenylalanine were found to produce an alteration of neural polyribosomes similar to that measured with phenylalanine. However, the injection of a balanced mixture of 6 or 7 neutral amino acids could restore the brain polyribosomes to normal states. Although this experimentally induced recovery did not lower brain phenylalanine concentrations, it did restore the acylation levels of methionyl-tRNA, and in particular, the methionyl-tRNA initiator species. This also led to a concomitant stimulation of the elongation rate of brain polypeptide synthesis. A natural recovery of brain polyribosomal levels (occurring 2 h after 1 mg/g phenylalanine is injected) did not appear to represent a real recovery of neural protein metabolism. Phenylalanine concentrations were increased in the brain, the acylation levels of methionyl-tRNA, alanyl-tRNA and the initiator methionyl-tRNA remained altered, and the rate of ribosome translocation was decreased 28%.

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Year:  1978        PMID: 245312     DOI: 10.1016/0005-2787(78)90214-9

Source DB:  PubMed          Journal:  Biochim Biophys Acta        ISSN: 0006-3002


  11 in total

1.  Abnormal amino acid metabolism and brain protein synthesis during neural development.

Authors:  J V Hughes; T C Johnson
Journal:  Neurochem Res       Date:  1978-08       Impact factor: 3.996

2.  In vitro localization of the protein synthesis defect associated with experimental phenylketonuria.

Authors:  M A Elsliger; G R Thériault; D Gauthier
Journal:  Neurochem Res       Date:  1989-01       Impact factor: 3.996

3.  Inhibition of cerebral protein kinase activity and cyclic AMP-dependent ribosomal-protein phosphorylation in experimental hyperphenylalaninaemia.

Authors:  S Roberts; B S Morelos
Journal:  Biochem J       Date:  1982-02-15       Impact factor: 3.857

4.  Decreased functional brain connectivity in individuals with early-treated phenylketonuria: evidence from resting state fMRI.

Authors:  Shawn E Christ; Amanda J Moffitt; Dawn Peck; Desirée A White; Joseph Hilgard
Journal:  J Inherit Metab Dis       Date:  2012-01-10       Impact factor: 4.982

5.  Characterization of cell-surface glycopeptides from mouse cerebral cortex that inhibit cell growth and protein synthesis.

Authors:  R J Kinders; A G Milenkovic; P Nordin; T C Johnson
Journal:  Biochem J       Date:  1980-09-15       Impact factor: 3.857

6.  Cerebral ribosomal protein phosphorylation in experimental hyperphenylalaninaemia.

Authors:  S Roberts; B S Morelos
Journal:  Biochem J       Date:  1980-08-15       Impact factor: 3.857

7.  Tract-based evaluation of white matter damage in individuals with early-treated phenylketonuria.

Authors:  Huiling Peng; Dawn Peck; Desirée A White; Shawn E Christ
Journal:  J Inherit Metab Dis       Date:  2013-09-17       Impact factor: 4.982

Review 8.  Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders.

Authors:  Dominic J Gessler; Guangping Gao
Journal:  Methods Mol Biol       Date:  2016

9.  The effects of chronic hyperphenylalaninaemia on mouse brain protein synthesis can be prevented by other amino acids.

Authors:  P Binek-Singer; T C Johnson
Journal:  Biochem J       Date:  1982-08-15       Impact factor: 3.857

10.  Brain uptake of 11C-methionine in phenylketonuria.

Authors:  D Comar; J M Saudubray; A Duthilleul; J Delforge; M Maziere; G Berger; C Charpentier; A Todd-Pokropek
Journal:  Eur J Pediatr       Date:  1981-03       Impact factor: 3.183

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