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

作者信息

Matsuo K, Hommes F

出版信息

J Inherit Metab Dis. 1987;10(1):62-5. doi: 10.1007/BF01799489.

DOI:10.1007/BF01799489
PMID:3033396
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.

摘要

相似文献

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引用本文的文献

1
White matter abnormalities in patients with treated hyperphenylalaninaemia: magnetic resonance relaxometry and proton spectroscopy findings.
Eur J Pediatr. 1993 Dec;152(12):1012-20. doi: 10.1007/BF01957228.

本文引用的文献

1
Neuropathological findings in phenyl-pyruvic oligophrenia (phenyl-ketonuria).苯丙酮尿性智力发育不全（苯丙酮尿症）的神经病理学发现。
J Neuropathol Exp Neurol. 1950 Jul;9(3):298-310. doi: 10.1097/00005072-195007000-00004.
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Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。
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PHENYLKETONURIA PRESENTING AN INTERMITTENT PROGRESSIVE COURSE.呈现间歇性进展病程的苯丙酮尿症。
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The association of phenylketonuria with leucodystrophy.苯丙酮尿症与脑白质营养不良的关联。
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Brain protein and myelin metabolism in hyperphenylalaninemic rats.高苯丙氨酸血症大鼠的脑蛋白和髓鞘代谢
Cell Mol Biol Incl Cyto Enzymol. 1980;26(1):31-6.
6
Turnover of the fast components of myelin and myelin proteins in experimental hyperphenylalaninaemia. Relevance to termination of dietary treatment in human phenylketonuria.实验性高苯丙氨酸血症中髓鞘及其蛋白快速成分的更新。与人类苯丙酮尿症饮食治疗的终止相关性。
J Inherit Metab Dis. 1982;5(1):21-7. doi: 10.1007/BF01799750.
7
Effect of experimental hyperphenylalaninemia on myelin metabolism at later stages of brain development.
Int J Neurosci. 1983 Sep;20(3-4):217-27. doi: 10.3109/00207458308986575.
8
Neuropathology of phenylketonuria.苯丙酮尿症的神经病理学
J Neuropathol Exp Neurol. 1966 Apr;25(2):254-68. doi: 10.1097/00005072-196604000-00006.
9
Effect of excess phenylalanine and of other amino acids on brain development in the infant rat.
Pediatr Res. 1970 Jan;4(1):96-102. doi: 10.1203/00006450-197001000-00012.
10
The protection of A1 myelin basic protein against the action of proteolytic enzymes after interaction of the protein with lipids at the air-water interface.在空气-水界面蛋白质与脂质相互作用后，A1髓鞘碱性蛋白对蛋白水解酶作用的保护作用。
Biochim Biophys Acta. 1973 Jul 18;311(4):520-30. doi: 10.1016/0005-2736(73)90127-2.