Abstract
Numerous “inborn errors” of amino acid metabolism have been described (15,22). Most of these are associated with clinical disease of varying severity. For example, while maple syrup urine disease is usually rapidly fatal (14) phenylketonuria is competible with relative longevity (10). In most instances, the inherited metabolic block in the amino acid(s) involved has been well defined. However, the pathogensis of the various clinical syndromes is less well delineated.
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References
Alvord, E. C., Stevenson, L. O., Vogel, E. S. and Egle, R. L.: Neuropathological findings in phenylpyruvic oligophrenia (phenyl-ketonuria).J. Neuropath. Exp. Neurol. 9, 298 (1950).
Aoki, K. and Seigel, F.L.: Hyperphenylalaninemia: disaggregation of brain polyribosomes in young rats. Science 168, 129 (1970).
Baglia, B. S., Pronczuk, A. W. and Munro, H. N.: Regulation of polysome aggregation in cell-free system through amino acid supply. J. Mole. Biol. 34, 199 (1968).
Baxter, C. F. and Tewari, S.: Regulation by amino acids of protein synthesis from immature rat brain: stimulatory effect of y-aminobutyric acid and glycine in Protein Metabolism of the Nervous System. Lajtha, A. (editor), New York, Plenum Press (1970) Page 439.
Brener, C. B. and Florini, A. J.: Amino acid incorporation into protein by cell-free system from rat skeletal muscle. IV. Biochemistry 4, 1544 (1965).
Chase, H. P. and O’Brien, D.: Effect of excess phenyalanine and of other amino acids on brain development in the infant rats. Pediat. Res. 4, 96 (1970).
Denckla, W. D. and Dewey, H. K.: The determination of trytophan in plasma, liver and urine. J. Lab. Clin. Med. 69, 160 (1967).
Ghadimi, H. and Pecora, P.: Free amino acids of cord plasma as compared with maternal plasma during pregnancy. Pediatrics 33, 500 (1964a).
Hsia, D. Y. Y.: Phenylketonuria: clinical, genetic and biochemical aspects. Warsaw Congress Proceedings of the International Association for Scientific Study of Mental Deficiency. (in press)
Lang, K.: Die phenylpyruvische oligophrenie. Ergebn, inn. Med. u. Kinderh. 6, 78 (1955).
Lowry, O. H., Rosebrough, N. J., Farr, A. L. and Randall, R. J.: Protein measurement with Folin phenol reagent. J. Biol. Chem. 193, 365 (1951).
Majumdar, C., Tsukada, K. and Liberman, I.: Liver protein synthesis after partial hepatectomy and acute stress. J. Biol. Chem 242, 700 (1967).
Medredev, Zh A.: Protein Biosynthesis and Problems of Heredity, Development and Ageing Edinburgh, Oliver and Boyd (1966).
Menkes, J. H., Hurst, P. L. and Craig, J. M.: A new syndrome: progressive familial cerebral dysfunction with an unusual urine substance Pediatrics 14, 462 (1954).
Nyhan, W. L. (editor): Amino Acid Metabolism and Genetic Variation, New York, McGraw-Hill Book Company (1967).
Oppenheim, J., Scheinbuks, J., Biava, C. and Marcus, L.: Polyribosomes in azotabacter vinelandii. Biochem. Biophys. Acta. 161, 386 (1968).
Orrego, F. and Lipmann, F.: Protein synthesis in brain slices. J. Biol. Chem. 242, 665 (1967).
Roberts, S. and Morelos, B. S.: Regulation of Cerebral metabolism of amino acid. J. Neurochem. 12, 373 (1965).
Siegel, F. L. Aoki, K. and Colwell, R. E.: Polyribosome disaggregation and cell-free protein synthesis in preparation from cortex of hyperphenylalaninemic rats. J. Neurochem. 18, 537 (1970).
Sox, H. C. and Hoagland, M. C.: Functional alteration in liver polysomes associated with starvation and refeeding. J. Molec. Biol. 20, 113 (1966).
Spackman, D. H., Stein, W. H. and Moore, S.: Automatic recording apparatus for use in chromatography of amino acids. Analytical Chem. 30, 1190 (1958).
Stanbury, J. B., Wyngaarden, J. B. and Fredrickson, D. S. (editors): The Metabolic Basis of Inherited Diseases. New York, McGraw-Hill Book Company (1966), pages 258–420.
Tewari, S. and Baxter, C. F.: Stimulatory effect of y-aminobutyric acid upon amino acid incorporation into protein by a ribosomal system from immature rat brain. J. Neurochem. 16, 171 (1969).
Tsukada, J., Majumdar, C. and Lieberman, I.: Liver polyribosomes and phospholipase A. Biochem. Biophys. Res. Commun. 25, 181 (1966).
Utosunomiya, T. and Roth, J. S.: Studies on the function of intracellular ribonucleases. J. Cell. Biol. 29, 395 (1966).
Winick, N.: Changes in nucleic acid and protein content of human brain during growth, Pediat. Res. 2, 352 (1968).
Winick, M. and Noble, A.: Quantitative changes in DNA, RNA and protein during prenatal and postnatal growth in rats. Develop. Biol. 12, 451 (1965).
Wunner, E. H., Bell, J. and Munro, H. N.: The effect of feeding with a trytophan-free amino acid mixture on rat liver polysomes and ribosomal ribonucleic acid. Biochem. J. 101, 417 (1966).
Zomzely, C. E., Roberts, S. and Rapoport, D.: Regulation of cerebral metabolism of amino acids. J. Neurochem. 11, 567 (1964).
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Wong, P.W.K., Justice, P. (1972). Effect of Amino Acid Imbalance on Polyribosome Profiles and Protein Synthesis in Fetal Cerebral Cortex. In: Volk, B.W., Aronson, S.M. (eds) Sphingolipids, Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 19. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6570-0_11
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DOI: https://doi.org/10.1007/978-1-4757-6570-0_11
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