Abstract
Experimental phenylketonuria was induced in newborn rats by administration ofl-phenylalanine and α-methylphenylalanine. There was no primary disturbance of myelination, but reduced cell proliferation, early cell death and compensatory hyperplasia were evident in the cerebellum.
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Berger, R., Springer, J. and Hommes, F. A. Brain protein and myelin metabolism in hyperphenylalaninemic rats.Cell Mol. Biol. 26 (1980) 31–36
Figlewicz, D. A. and Druse, M. J. Experimental hyperphenylalaninaemia: effect on central nervous system myelin subfractions.Exp. Neurol. 67 (1980) 315–329
Greengard, O., Yoss, M. S. and DelValle, J. A. α-Methylphenylalanine, a new inducer of chronic hyperphenylalaninemia in suckling rats.Science 192 (1976) 1003–1008
Huether, G., Kaus, R. and Neuhoff, V. Brain development in experimental hyperphenylalaninemia: myelination.Exp. Neurol. (submitted, 1981a)
Huether, G., Kaus, R. and Neuhoff, V. Brain development in experimental hyperphenylalaninemia: disturbed proliferation and reduced cell numbers in the cerebellum.Brain Research (submitted, 1981b)
Hughes, J. V. and Johnson, T. C. Abnormal amino acid metabolism and brain protein synthesis during neural development.Neurochem. Res. 3 (1978) 381–399
Lane, J. D. and Neuhoff, V. Phenylketonuria. Clinical and experimental considerations revealed by the use of animal models.Naturwissenschaften 67 (1980) 227–233
Lane, J. D., Schöne, B., Langenbeck, U. and Neuhoff, V. Characterization of experimental phenylketonuria. Augmentation of hyperphenylalaninemia with α-methylphenylalanine andp-chlorophenylalanine.Biochim. Biophys. Acta 627 (1980) 114–156
Linneweh, F. and Ehrlich, M. Zur Pathogenese des Schwachsinns bei PKU.Klin. Wochenschr. 40 (1962) 225–226
Lipton, M. A., Gordon, R., Guroff, G. and Udenfriend, S. Parachlorophenylalanine induced chemical manifestations of phenylketonuria in rats.Science 156 (1967) 248–250
Norton, W. T. and Poduslo, S. E. Myelination in rat brain: method of myelin isolation.J. Neurochem. 21 (1973) 749–757
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Huether, G., Neuhoff, V. Use of α-methylphenylalanine for studies of brain development in experimental phenylketonuria. J Inherit Metab Dis 4, 67–68 (1981). https://doi.org/10.1007/BF02263594
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DOI: https://doi.org/10.1007/BF02263594