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Regional distribution of the phenylalanine-sensitive ATP-sulphurylase in brain

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The regional distribution of the phenylalanine-sensitive ATP-sulphurylase in fetal calf brain coincides with demyelinated lesions observed in the central nervous systems of untreated PKU patients. This would be expected if this species of ATP-sulphurylase played a role in the pathogenesis of brain dysfunction in the untreated or poorly controlled phenylketonuria patient.

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  1. Alvord, E. C., Stevenson, L. D., Vogel, F. S. and Engle, R. L. Neuropathological findings in phenyl pyruvic oligophrenia (phenylketonuria).J. Neuropathol. Exp. Neurol. 9 (1950) 298–310

  2. Bechar, M., Borstein, B., Elion, M. and Sandbank, U. Phenylketonuria presenting an intermittant progressive course.J. Neurol. Neurosurg. Psychiatry 28 (1965) 165–170

  3. Berger, R., Springer, J. and Hommes, F. A. Brain protein and myelin metabolism in young hyperphenylalaninemic rats.Mol. Cell. Biol. 26 (1980) 31–36

  4. Chase, H. P. and O'Brien, D. Effect of excess phenylalanine and of other amino acids on brain development in the infant rat.Pediatr. Res. 4 (1970) 96–102

  5. Crome, L. The association of phenylketonuria with leukodystrophy.J. Neurol. Neurosurg. Psychiatry 25 (1962) 149–153

  6. Crome, L. The morbid anatomy of phenylketonuria. In Bickel, H., Hudson, F. P. and Woolf, L. I. (eds.)Phenylketonuria and some other Inborn Errors of Amino Acid Metabolism, Thieme, Stuttgart, 1971, pp. 126–131

  7. Grundt, I. and Hole, K.p-Chlorophenylalanine treatment in developing rats: protein and lipids in whole brain and myelin.Brain Res. 74 (1974) 269–277

  8. Hommes, F. A. Myelin turnover at later stages of brain development in experimental hyperphenylalaninemia. In Bickel, H. and Wachtel, U. (eds.)Inherited Diseases of Amino Acid Metabolism, Recent Progress in the Understanding, Recognition and Management, Thieme, Stuttgart, 1985a, pp. 67–85

  9. Hommes, F. A. Amino acidemias and brain maturation. Interference with sulfate activation and myelin metabolism.J. Inher. Metab. Dis. 8, Suppl. 2 (1985b) 121–122

  10. Hommes, F. A., Eller, A. G. and Taylor, E. H. Turnover of the fast component of myelin and myelin proteins in experimental hyperphenylalaninemia. Relevance to termination of dietary treatment of PKU.J. Inher. Metab. Dis. 5 (1982) 21–27

  11. Hommes, F. A. and Moss, L. The assay of ATP-sulfurylase.Anal. Biochem. 154 (1986) 100–104

  12. London, Y., Demel, R. A., Geurts van Kessel, W. S. M., Vossenberg, F. G. A. and Van Deenen, L. L. M. The protection of A1 myelin basic protein against the action of proteolytic enzymes after interaction of the protein with lipids at the air-water interphase.Biochim. Biophys. Acta 311 (1973) 520–530

  13. Lowry, O. H., Rosebrough, N., Farr, L. A. and Randall, R. J. Protein measurement with the Folin phenol reagent.J. Biol. Chem. 193 (1951) 265–275

  14. Malamud, N. Neuropathology of phenylketonuria.J. Neuropathol. Exp. Neurol. 25 (1966) 254–268

  15. Matsuo, K., Moss, L. and Hommes, F. A. Properties of the 3′-phosphoadenosine-5′-phosphosulfate (PAPS) synthesizing systems of brain and liver.Neurochem. Res., in press

  16. Shah, S. N., Peterson, N. A. and McKean, C. M. Lipid composition of human cerebral white matter and myelin in phenylketonuria.J. Neurochem. 19 (1972) 2369–2376

  17. Sprinkle, T. and Rennert, O. M. The effect of phenylketonuria and other metabolites on sulfated galactocerebroside synthesisin vivo and in culture.J. Neurochem. 26 (1976) 499–504

  18. Taylor, E. H. and Hommes, F. A. Effect of experimental hyperphenylalaninemia on myelin metabolism at later stages of brain development.Int. J. Neurosci. 20 (1983) 217–228

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Matsuo, K., Hommes, F. Regional distribution of the phenylalanine-sensitive ATP-sulphurylase in brain. J Inherit Metab Dis 10, 62–65 (1987).

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  • Public Health
  • Nervous System
  • Central Nervous System
  • Internal Medicine
  • Metabolic Disease