Skip to main content
SpringerLink
Log in

Experimental Aminoacidemias

  • Chapter
Alterations of Metabolites in the Nervous System

  • 44 Accesses

Abstract

Abnormalities involving the human central nervous system in prenatal or early postnatal life have been described in the presence of derangements in the metabolism of several amino acids, including phenylalanine, tyrosine, branched-chain amino acids, histidine, glycine, lysine, proline, methionine, and homocystine.1–5 The roster of known amino acid dyscrasias associated with brain damage has increased almost annually over the past decade and appears to be far from complete. These diseases are generally of genetic origin. However, experimental studies suggest that environmental factors contribute to the expression of these dyscrasias and may, indeed, be responsible for their occurrence in certain cases.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Gaull, G. E., Tallan, H. H., Lajtha, A., and Rassin, D. K.. 1975, Biology of Brain Dysfunction, Volume 3 (G. E. Gaull, ed.), Plenum Press, New York. pp. 47–143.

    Chapter  Google Scholar 

  2. Kaufman, S., 1977, Advances in Neurochemistry, Volume 2 (B. W. Agranoff and M. H. Aprison, eds.). Plenum Press, New York, pp. 1–132.

    Google Scholar 

  3. Rosenberg, L. E., and Scriver, C. R., 1980, Metabolic Control and Disease. 8th ed. ( P. K. Bondy and L. E. Rosenberg, eds.), W. B. Saunders, Philadelphia, pp. 583–776.

    Google Scholar 

  4. Nyhan, W. L., 1981, Comprehensive Biochemistry: Amino Acid Metabolism and Sulphur Metabolism, Volume 19A (A. Neuberger, ed.), Elsevier, Amsterdam, pp. 305–468.

    Google Scholar 

  5. Stanbury, J. B., Wyngaarden, J. B., Fredrickson. D. S., Goldstein, J. L., and Brown, M. S. (eds.), 1983, The Metabolic Basis of Inherited Disease, 5th ed., McGraw-Hill, New York.

    Google Scholar 

  6. Holtzman, N. A.. 1970, Annie. Rev. Med. 21: 335–356.

    Article  CAS  Google Scholar 

  7. Roberts, S., Seto. K.. and Hanking, B. H., 1962, J. Neurochem. 9: 493–501.

    Article  PubMed  CAS  Google Scholar 

  8. Harper, A. E., Benevenga, N. J., and Wohlhueter, R. M., 1970, Physiol. Rev. 50: 428–558.

    PubMed  CAS  Google Scholar 

  9. Fernstrom, J. D., 1977,;Metabolism 26: 207–223.

    Google Scholar 

  10. Zamenhof, S., and van Marthens, E., 1978, Early Influences: Studies on the Development of Behavior and the Nervous System, Volume 4 (G. Gottlieb, ed.), Academic Press, New York, pp. 149–186.

    Google Scholar 

  11. Vorhees, C. V., Butcher, R. E., and Berry, H. K., 1981, Neurosci. Behay. Rev. 5:177–190.

    Article  CAS  Google Scholar 

  12. DelValle, J. A., Dienel, G., and Greengard, O., 1978, Biochem. J. 170: 449–459.

    PubMed  CAS  Google Scholar 

  13. McKean, C. M., Boggs, D. E., and Peterson, N. A., 1968, J. Neurochern. 15: 235–241.

    Article  CAS  Google Scholar 

  14. McKean, C. M., 1972, Brain Res. 47: 469–476.

    Article  PubMed  CAS  Google Scholar 

  15. Roberts, S., and Morelos, B. S., 1976, J. Neurochem. 26: 387–400.

    Article  PubMed  CAS  Google Scholar 

  16. Isaacs, C. E., and Greengard, O., 1980, Biochem. J. 192: 441–448.

    PubMed  CAS  Google Scholar 

  17. Dienel, G. A., 1981, J. Neurochern. 36: 34–43.

    Article  CAS  Google Scholar 

  18. Waisman, H. A., Wang, H. L., Palmer, G., and Harlow, H. F., 1960, Nature 188: 1124–1125.

    Article  PubMed  CAS  Google Scholar 

  19. Brunner, R. L., Vorhees, C. V., McLean, M. S., Butcher, R. E., and Berry, H. K.. 1978, Brain Res. 154: 191–195.

    Article  PubMed  CAS  Google Scholar 

  20. Pease, D. C. (ed.), 1971, Cellular Aspects of Neural Growth and Differentiation, UCLA Forum in Medical Sciences No. 14, University of California Press, Los Angeles.

    Google Scholar 

  21. Chirigos, M. A., Greengard, P., and Udenfriend, S., 1960, J. Biol. Chem. 235: 2075–2079.

    PubMed  CAS  Google Scholar 

  22. Lajtha, A., and Toth, J., 1961, J. Neurochem. 8: 216–225.

    Article  PubMed  CAS  Google Scholar 

  23. Guroff, G., and Udenfriend, S., 1962, J. Biol. Chem. 237: 803–806.

    PubMed  CAS  Google Scholar 

  24. Roberts, S., and Morelos, B. S., 1965, J. Neurochem. 12: 373–387.

    Article  PubMed  CAS  Google Scholar 

  25. Roberts, S., 1968, Progress in Brain Research. Volume 29 (A. Lajtha and D. H. Ford, eds.). Elsevier, Amsterdam, pp. 235–243.

    Google Scholar 

  26. Lajtha, A., 1974, Aromatic Amino Acids in the Brain, Ciba Foundation Symposium 22 (New Series), Elsevier/Excerpta MedicaiNorth-Holland, Amsterdam, pp. 25–49.

    Google Scholar 

  27. Oldendorf, W. H., 1973, Arch. Neurol. 28: 45–48.

    Article  PubMed  CAS  Google Scholar 

  28. Pardridge, W. M., 1977, Determinants of the Availability of Nutrients to the Brain, Volume 1 (R. J. Wurtman and J. J. Wurtman, eds.), Raven Press, New York. pp. 141–204.

    Google Scholar 

  29. McKean, C. M., Schanberg, S. M., and Giarman, N. J., 1962. Science 137: 604–605.

    Article  PubMed  CAS  Google Scholar 

  30. Aoki, K., and Siegel, F. L., 1970, Science 168: 129–130.

    Article  PubMed  CAS  Google Scholar 

  31. Yuwiler, A., and Louttit, R. T., 1961. Science 134: 831–832.

    Article  PubMed  CAS  Google Scholar 

  32. Weiss, B. F., Munro, H. N., Ordonez, L. A., and Wurtman, R. J., 1972. Science 177: 613–616.

    Article  PubMed  CAS  Google Scholar 

  33. Munro, H. N., Roel, L. E., and Wurtman, R. J., 1973, J. Neural Transm. 34: 321–323.

    Article  PubMed  CAS  Google Scholar 

  34. Carver, M. J., 1969, J. Neurochem. 16: 113–116.

    Article  PubMed  CAS  Google Scholar 

  35. O’Brien, D., and Ibbot. F. A., 1966, Dev. Med. Child. Neurol. 8: 724–728.

    Article  PubMed  Google Scholar 

  36. Roberts, S., 1974, Aromatic Amino Acids in the Brain: Ciba Foundation Symposium 22 (New Series),Elsevier/Excerpta Medica/North-Holland, Amsterdam, pp. 299–318.

    Google Scholar 

  37. Daniel, R. G., and Waisman, H. A., 1969, J. Neurochem. 16: 787–795.

    Article  PubMed  CAS  Google Scholar 

  38. Fando, J. L.. Domínguez. F.. and Herrera, E., 1981, J. Neurochem. 37: 824–829.

    Article  PubMed  CAS  Google Scholar 

  39. Neame. K. D., 1961. Nature 192: 173–174.

    Article  PubMed  Google Scholar 

  40. Blasberg, R.. and Lajtha, A., 1966, Brain Res. 1: 86–104.

    PubMed  CAS  Google Scholar 

  41. Hughes, J. V., and Johnson. T. C., 1976, J. Neurochem. 26: 1105–1113.

    Article  PubMed  CAS  Google Scholar 

  42. Roberts. S. Zomzely, C. E., and Bondy, S. C., 1971, Cellular Aspects of Neural Growth and Differentiation, UCLA Forum in Medical Sciences No. 14 (D. C. Pease, ed.), University of California Press, Los Angeles, pp. 447–471.

    Google Scholar 

  43. Benuck, M., and Lajtha, A.. 1975, Int. Rev. Neurobiol. 17: 85–129.

    Article  PubMed  CAS  Google Scholar 

  44. Zomzely. C. E., Roberts, S.. and Rapaport, D., 1964, J. Neurochem. 11: 567–582.

    Article  Google Scholar 

  45. Appel, S. H.. 1966, Trans. N.Y. Acad. Sci. 29: 63–70.

    Article  PubMed  CAS  Google Scholar 

  46. Campagnoni, A. T., and Mahler. H. R., 1967, Biochemistry 6: 956–967.

    Article  PubMed  CAS  Google Scholar 

  47. Maclnnes, J. W., and Schlesinger, K., 1971, Brain Res. 29: 101–110.

    Article  Google Scholar 

  48. Oja. S. S., Landesmaki, P.. and Vahvelainen, M.-L., 1974, Aromatic Amino Acids in the Brain, Ciba Foundation Symposium 22 (New Series), Elsevier/Excerpta Medica/North-Holland, Amsterdam, pp. 283–297.

    Google Scholar 

  49. Bogucki, B., and Roberts, S., 1979, Fed. Proc. 38: s947.

    Google Scholar 

  50. Dunlop, D. S., van Elden, W., and Lajtha, A., 1974, J. Neurochem. 22: 821–830.

    Article  PubMed  CAS  Google Scholar 

  51. Swaiman, K. F.. Hosfield, W. B.. and Lemieux. B., 1968, J. Neurochem. 15: 687–690.

    Article  PubMed  CAS  Google Scholar 

  52. Lo.. G. S., Lee. S., Cruz. N. L.. and Longenecker, J. B., 1970. Nutr. Rep. Int. 2: 59–72.

    Google Scholar 

  53. Lindroos, O. F. C., and Oja. S. S., 1971, Exp. Brain Res. 14: 48–60.

    Article  PubMed  CAS  Google Scholar 

  54. Mitoma, C., and LeValley, S. E., 1973, Proc. Soc. Exp. Biol. Med. 144: 710–713.

    Article  PubMed  CAS  Google Scholar 

  55. Weiss, B. F.. Roel, L. E., Munro, H. N., and Wurtman, R. J., 1974, Aromatic Amino Acids in the Brain, Ciba Foundation Symposium 22 (New Series),Elsevier/Excerpta Medica/NorthHolland, pp. 325–334.

    Google Scholar 

  56. Dunlop, D. S., van Elden, W., and Lajtha, A., 1975, J. Neurochem. 24: 337–344.

    Article  PubMed  CAS  Google Scholar 

  57. Agrawal, H. C., Bone, A. H., and Davison, A. N., 1970, Biochem. J. 117: 325–331.

    PubMed  CAS  Google Scholar 

  58. Figlewicz. D. A., and Druse, M. J., 1980, Exp. Neurol. 67: 315–329.

    Article  Google Scholar 

  59. Siegel, F. L., Aoki, K., and Colwell, R. E., 1971, J. Neurochem. 18: 537–547.

    Article  PubMed  CAS  Google Scholar 

  60. Taub. F., and Johnson, T. C., 1975, Biochem. J. 151: 173–180.

    Google Scholar 

  61. Wong, P. W. K.. Fresco, R., and Justice, P., 1972, Metabolism 21: 875–881.

    Article  PubMed  CAS  Google Scholar 

  62. Hughes, J. V., and Johnson, T. C., 1978, Biochim. Biophys. Acta 517: 473–485.

    Article  PubMed  CAS  Google Scholar 

  63. Roberts, S., 1977, Mechanisms, Regulation and Special Functions of Protein Synthesis in the Brain (S. Roberts, A. Lajtha, and W. H. Gispen, eds.), Elsevier/North-Holland Biomedical Press, Amsterdam, pp. 3–20.

    Google Scholar 

  64. Roberts, S., 1982, Brain Phosphoproteins: Characterization and Functions (W. H. Gispen and A. Routtenberg. eds.), Frog. Brain Res. 56: 195–211.

    Google Scholar 

  65. Roberts. S., and Morelos, B. S., 1980, Biochem. J. 190: 405–419.

    Google Scholar 

  66. Weber, G., Glazer, R. 1.. and Ross, R. A., 1970, Adv. Enzyme Regal. 8: 13–36.

    Article  CAS  Google Scholar 

  67. Dwivedy, A. K., and Shah. S. N., 1982, Neurochem. Res. 7: 717–725.

    Article  PubMed  CAS  Google Scholar 

  68. Patel, M. S., 1972, Biochem. J. 128: 677–684.

    PubMed  CAS  Google Scholar 

  69. Land, J. M.. and Clark, J. B., 1974, FEBS Lett. 44: 348–351.

    Article  PubMed  CAS  Google Scholar 

  70. Benavides, J., Gimenez, C., Valdivieso, F., and Mayor, F., 1976, Biochem. J. 160: 217–222.

    PubMed  CAS  Google Scholar 

  71. Davison. A. N.. and Sandler, M., 1958, Nature 181: 186–187.

    Article  Google Scholar 

  72. Hughes. J. V., and Johnson, T. C., 1977, Biochem. J. 162: 527–537.

    Google Scholar 

  73. Binek, P. A., Johnson, T. C., and Kelly, C. J.. 1981. J. Neurochem. 36: 1476–1484.

    Article  PubMed  CAS  Google Scholar 

  74. Binek-Singer, P., and Johnson. T. C., 1982, Biochem. J. 206: 407–414.

    PubMed  CAS  Google Scholar 

  75. Neuberger, A., and Tatum, E. L. (eds.), 1969, 1973, 1975, 1976, 1979, Lysosomes in Biology and Pathology. Volumes 1–6, North-Holland, Amsterdam.

    Google Scholar 

  76. Huether, G., and Neuhoff. V., 1981, J. Inherit. Metab. Dis. 4: 67–68.

    Article  PubMed  CAS  Google Scholar 

  77. Lloyd, J. B., 1971, Biochem. J. 121: 245–248.

    PubMed  CAS  Google Scholar 

  78. Albrecht, J., and Yanagihara. T.. 1979, J. Neurochem. 32: 1131–1133.

    Article  PubMed  CAS  Google Scholar 

  79. Himwich, H. E., and Fazekas, J. F., 1940, Arch. Neurol. Psychiatry 44: 1213–1218.

    Article  Google Scholar 

  80. Einstein. E. R., Csejtey, J., Dalal, K. B., Adams. C. W. M., Bayliss. O. B., and Hallpike, J. F.. 1972. J. Neurochem. 19: 653–662.

    Article  Google Scholar 

  81. Hirsch, H. E.. and Parks. M. E., 1975, J. Neurochem. 24: 853–858.

    Article  PubMed  CAS  Google Scholar 

  82. Adams, C. W. M., 1972, Research in Multiple Sclerosis (W. M. Adams, ed.. with a contribution by S. Liebowitz), Charles C Thomas, Springfield, Illinois. pp. 61–134.

    Google Scholar 

  83. Hartmann, J. F., and Becker, R. A., 1973, J. Neural Transrn. 34: 73–77.

    Article  CAS  Google Scholar 

  84. Ochoa, S., and de Haro, C., 1979. Anna. Rev. Biochem. 48: 549–580.

    Article  CAS  Google Scholar 

  85. Holbrook, L. A., and Roberts, S., 1979, Soc. Neurosci. Abstr. 5: 405.

    Google Scholar 

  86. Roberts, S., and Morelos, B. S., 1982. Biochem. J. 202: 343–351.

    PubMed  CAS  Google Scholar 

  87. Wool, I. G., 1979, Anna. Rev. Biochem. 48: 719–754.

    Article  CAS  Google Scholar 

  88. Traugh, J. A., 1981, Biochemical Actions of Hormones, Volume 8 (G. Litwack. ed.). Academic Press, New York. pp. 167–208.

    Google Scholar 

  89. Leader, D. P., and Coia, A. A., 1978, FEBS Leu. 90: 270–274.

    Article  CAS  Google Scholar 

  90. Schubart, U. K., Shapiro. S., Fleicher, N.. and Rosen, O. M., 1977. J. Biol. Chem. 252: 92101.

    Google Scholar 

  91. Martini, O. H. W., and Kruppa, J., 1979, Eur. J. Biochern. 95: 349–358.

    Article  CAS  Google Scholar 

  92. Wettenhall, R. E. H., and Howlett, G. J., 1979, J. Biol. Chem. 254: 9317–9323.

    PubMed  CAS  Google Scholar 

  93. Thomas, A., Spaan, W., van Steeg, H., Voorma, H. O.. and Benne. R., 1980, FEBS Lett. 116: 67–71.

    Article  PubMed  CAS  Google Scholar 

  94. Terao, K., and Ogata, K., 1979, J. Biochem. 86: 597–603.

    PubMed  CAS  Google Scholar 

  95. Terao, K., and Ogata, K., 1979, J. Biochem. 86: 605–617.

    PubMed  CAS  Google Scholar 

  96. Stahl, J., and Kobets, N. D., 1981, FEBS Lett. 123: 269–272.

    Article  PubMed  CAS  Google Scholar 

  97. Lastick, S. M., and McConkey, E. H., 1981. J. Biol. Chem. 256: 583–585.

    PubMed  CAS  Google Scholar 

  98. Roberts, S., and Morelos, B. S., 1979, Biochem. J. 184: 233–244.

    PubMed  CAS  Google Scholar 

  99. Walter, U., and Greengard, P., 1981, Current Topics in Cellular Regulation. Volume 19 (B. L. Horecker and E. R. Stadtman, eds.). Academic Press. New York. pp. 219–256.

    Google Scholar 

  100. Lohmann, S. M.. Walter, U., and Greengard, P., 1980, J. Biol. Chem. 255: 9985–9992.

    PubMed  CAS  Google Scholar 

  101. Krebs, E. G., and Beavo, J. A., 1979, Anna. Rev. Biochem. 48: 923–959.

    Article  CAS  Google Scholar 

  102. Patel, M. S., and Arinze, I. J., 1975, Am. J. Clin. Nair. 28: 183–188.

    CAS  Google Scholar 

  103. McKean, C. M., and Peterson, N. A., 1970, N. Engl. J. Med. 283: 1364–1367.

    Article  PubMed  CAS  Google Scholar 

  104. Partington, M. W., and Vickery, S. K., 1974, Neuropaediatrie 5: 125–137.

    Article  CAS  Google Scholar 

  105. Land, J. M., and Clark, J. B., 1973, Biochem. J. 134: 545–555.

    PubMed  CAS  Google Scholar 

  106. Land, J. M.. Mowbray, J., and Clark, J. B.. 1976. J. Neurochem. 26: 823–830.

    Article  PubMed  CAS  Google Scholar 

  107. Loo, Y. H., Scotto, L., and Horning, M. G.. 1977. J. Neurochem. 29: 411–415.

    Article  PubMed  CAS  Google Scholar 

  108. Silberberg, D. H., 1969, J. Neurochem. 16: 1141–1146.

    Article  PubMed  CAS  Google Scholar 

  109. Patel, M. S., and Owen. O. E., 1978, J. Neurochem. 30: 775–782.

    Article  PubMed  CAS  Google Scholar 

  110. Clark, J. B., and Land, J. M., 1974, Biochem. J. 140: 25–29.

    PubMed  CAS  Google Scholar 

  111. Williamson, D. H., and Buckley, B. M., 1973, Inborn Errors of Metabolism (F. A. Hommes and C. J. van den Berg), Academic Press. New York, pp. 81–96.

    Google Scholar 

  112. Edmond, J., 1974, J. Biol. Chem. 249: 72–80.

    PubMed  CAS  Google Scholar 

  113. Dreyfus, P. M., and Prensky, A. L.. 1967, Nature 214: 276.

    Article  PubMed  CAS  Google Scholar 

  114. Cremer, J. E., Teal, H. M., and Cunningham. V. J., 1982. J. Neurochem. 39: 674–677.

    Article  PubMed  CAS  Google Scholar 

  115. Miller, A. L., Hawkins, R. A., and Veech, R. L., 1973. Science 173: 904–906.

    Article  Google Scholar 

  116. Gimenez, C., Valdivieso, F., and Mayor. F., 1974, Biochem.;Lied. 11: 81–86.

    CAS  Google Scholar 

  117. Wapnir, R. A., Moak, S. A.. and Lifshitz, F., 1977, Nature 265: 647–648.

    Article  PubMed  CAS  Google Scholar 

  118. Akeson, A. L.. Berry, H. K., Brunner, R. L.. and Vorhees. C. V.. 1979, J. Neurochem. 32: 233–235.

    Article  PubMed  CAS  Google Scholar 

  119. Prensky, A. L., Fishman, M. A., and Daftari, B., 1971, Brain Res. 33: 181–191.

    Article  PubMed  CAS  Google Scholar 

  120. Anderson, A. E.. and Guroff, G., 1972, Proc. Natl. Acad. Sci. U.S.A. 69: 863–867.

    Article  Google Scholar 

  121. Loo, Y. H., Scotto, J., and Wiesniewski, H. M., 1978, Mvelination and Demyelination (J. Palo, ed.), Plenum Press, New York, pp. 453–469.

    Chapter  Google Scholar 

  122. Fulton, T. R., Triano, T.. Rabe, A., and Loo, Y. H., 1980, Life Sci. 27: 1271–1281.

    Article  PubMed  CAS  Google Scholar 

  123. Lane, J. D., and Neuhoff, V., 1980, Naturwissenschaften 67: 227–233.

    Article  PubMed  CAS  Google Scholar 

  124. Nigam, M. P., and Labar, D. R., 1979, Brain Res. 179: 195–198.

    Article  PubMed  CAS  Google Scholar 

  125. Hogan, R. N., and Coleman, P. D., 1981, Exp. Neurol. 74: 218–233.

    Article  PubMed  CAS  Google Scholar 

  126. Hogan, R. N., and Coleman, P. D., 1981, Exp. Nearol. 74: 234–244.

    Article  CAS  Google Scholar 

  127. Shah, S. N., Peterson, N. A.. and McKean. C. M.. 1972, J. Neurochem. 19: 2369–2376.

    Article  PubMed  CAS  Google Scholar 

  128. Johnson, R. C., and Shah, S. N., 1980, Neurochem. Res. 5: 709–718.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Biological Chemistry, School of Medicine, and the Brain Research Institute, UCLA Center for the Health Sciences, Los Angeles, California, 90024, USA

    Sidney Roberts

Authors
  1. Sidney Roberts
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Center for Neurochemistry, Wards Island, New York, USA

    Abel Lajtha

Rights and permissions

Reprints and permissions

Copyright information

© 1985 Springer Science+Business Media New York

About this chapter

Cite this chapter

Roberts, S. (1985). Experimental Aminoacidemias. In: Lajtha, A. (eds) Alterations of Metabolites in the Nervous System. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6740-7_8

Download citation

  • DOI: https://doi.org/10.1007/978-1-4757-6740-7_8

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4757-6742-1

  • Online ISBN: 978-1-4757-6740-7

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation