Abstract
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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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
Bechar, M., Borstein, B., Elion, M. and Sandbank, U. Phenylketonuria presenting an intermittant progressive course.J. Neurol. Neurosurg. Psychiatry 28 (1965) 165–170
Berger, R., Springer, J. and Hommes, F. A. Brain protein and myelin metabolism in young hyperphenylalaninemic rats.Mol. Cell. Biol. 26 (1980) 31–36
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
Crome, L. The association of phenylketonuria with leukodystrophy.J. Neurol. Neurosurg. Psychiatry 25 (1962) 149–153
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
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
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
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
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
Hommes, F. A. and Moss, L. The assay of ATP-sulfurylase.Anal. Biochem. 154 (1986) 100–104
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
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
Malamud, N. Neuropathology of phenylketonuria.J. Neuropathol. Exp. Neurol. 25 (1966) 254–268
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
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
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
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). https://doi.org/10.1007/BF01799489
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DOI: https://doi.org/10.1007/BF01799489