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Inhibition of brain glutamic acid decarboxylase by phenylalanine, valine, and leucine derivatives: a suggestion concerning the etiology of the neurological defect in phenylketonuria and branched-chain ketonuria.

作者信息

TASHIAN R E

出版信息

Metabolism. 1961 May;10:393-402.

PMID:13775398
Abstract
摘要

相似文献

1
Inhibition of brain glutamic acid decarboxylase by phenylalanine, valine, and leucine derivatives: a suggestion concerning the etiology of the neurological defect in phenylketonuria and branched-chain ketonuria.苯丙氨酸、缬氨酸和亮氨酸衍生物对脑谷氨酸脱羧酶的抑制作用：关于苯丙酮尿症和支链酮尿症神经缺陷病因的一种推测。
Metabolism. 1961 May;10:393-402.
2
The need of essential amino acids in children. An evaluation based on the intake of phenylalanine, tyrosine, leucine, isoleucine, and valine in children with phenylketonuria, tyrosine amino transferase defect, and maple syrup urine disease.儿童对必需氨基酸的需求。基于苯丙酮尿症、酪氨酸氨基转移酶缺陷症和枫糖尿症患儿苯丙氨酸、酪氨酸、亮氨酸、异亮氨酸和缬氨酸摄入量的评估。
Am J Clin Nutr. 1980 Feb;33(2):279-86. doi: 10.1093/ajcn/33.2.279.
3
Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency.枫糖尿症；一种与严重智力缺陷相关的缬氨酸、亮氨酸和异亮氨酸代谢先天性缺陷。
Br Med J. 1959 Jan 10;1(5114):90-1. doi: 10.1136/bmj.1.5114.90.
4
[CLINICAL, BIOCHEMICAL, AND NEUROPATHOLOGIC ASPECTS OF MAPLE SYRUP URINE DISEASE. A KETOACIDURIA OF THE OPEN CHAIN AMINOACIDS LEUCINE, ISOLEUCINE AND VALINE].
An Fac Med Lima. 1963 Sep;46:339-65.
5
THE DIAGNOSIS OF MAPLE SYRUP URINE DISEASE (BRANCHED- CHAIN KETOACIDURIA) BY THE IN VITRO STUDY OF THE PERIPHERAL LEUKOCYTE.通过外周血白细胞的体外研究诊断枫糖尿症（支链酮酸尿症）
Pediatrics. 1963 Aug;32:234-8.
6
[Pathogenetic aspects of some congenital errors of metabolism].
Pediatria (Napoli). 1962 May-Jun;70:x-xvi contd.
7
[Intermittent branched--chain ketoacidurie in ketotic hypoglycemia: investigations to localize the biochemical defect (author's transl)].
Monatsschr Kinderheilkd (1902). 1976 Feb;124(2):59-65.
8
THE PRIMARY AMINO-ACIDOPATHIES. GENETIC DEFECTS IN THE METABOLISM OF THE AMINO ACIDS.原发性氨基酸病。氨基酸代谢中的遗传缺陷。
Pediatr Clin North Am. 1963 Aug;10:723-44. doi: 10.1016/s0031-3955(16)31448-1.
9
Inhibition of DOPA decarboxylase by aromatic acids associated with phenylpyruvic oligophrenia.
Proc Soc Exp Biol Med. 1956 Dec;93(3):413-4. doi: 10.3181/00379727-93-22773.
10
Phenylketonuria: metabolic alterations induced by phenylalanine and phenylpyruvate.苯丙酮尿症：苯丙氨酸和苯丙酮酸引起的代谢改变。
Am J Clin Nutr. 1975 Feb;28(2):183-8. doi: 10.1093/ajcn/28.2.183.

引用本文的文献

1
Maple syrup urine disease: An uncommon cause for neonatal metabolic distress.枫糖尿症：新生儿代谢窘迫的罕见病因。
Indian J Clin Biochem. 1999 Jul;14(2):198-206. doi: 10.1007/BF02867919.
2
The intra-hippocampal leucine administration impairs memory consolidation and LTP generation in rats.内侧海马内亮氨酸给药可损害大鼠的记忆巩固和 LTP 产生。
Cell Mol Neurobiol. 2010 Oct;30(7):1067-75. doi: 10.1007/s10571-010-9538-4. Epub 2010 Jun 26.
3
Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease.
枫糖尿症中积累的支链氨基酸对脑能量代谢的抑制作用。
Neurochem Res. 2008 Jan;33(1):114-24. doi: 10.1007/s11064-007-9423-9. Epub 2007 Aug 8.
4
Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.细胞骨架作为枫糖尿症神经病理学的潜在靶点：来自动物研究的见解
J Inherit Metab Dis. 2007 Oct;30(5):664-72. doi: 10.1007/s10545-007-0562-6. Epub 2007 Jun 14.
5
Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.肌酸和抗氧化剂治疗可预防枫糖尿症中积累的支链α-酮酸所诱导的肌酸激酶活性抑制及C6胶质瘤细胞的形态学改变。
Cell Mol Neurobiol. 2006 Feb;26(1):67-79. doi: 10.1007/s10571-006-9098-9.
6
Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.枫糖尿症中积累的支链α-酮酸诱导C6胶质瘤细胞中磷酸化胶质纤维酸性蛋白的重新组织。
Metab Brain Dis. 2005 Sep;20(3):205-17. doi: 10.1007/s11011-005-7208-x.
7
Morphological alterations and cell death provoked by the branched-chain alpha-amino acids accumulating in maple syrup urine disease in astrocytes from rat cerebral cortex.枫糖尿症中支链α-氨基酸在大鼠大脑皮质星形胶质细胞中积累所引发的形态学改变和细胞死亡。
Cell Mol Neurobiol. 2005 Aug;25(5):851-67. doi: 10.1007/s10571-005-4938-6.
8
Importance of choline during growth, with particular reference to synthetic diets in phenylketonuria.胆碱在生长过程中的重要性，尤其涉及苯丙酮尿症的合成饮食。
Arch Dis Child. 1962 Dec;37(196):565-77. doi: 10.1136/adc.37.196.565.
9
Trial of amino-oxyacetic acid, an anticonvulsant.抗惊厥药氨基氧乙酸的试验
Can Med Assoc J. 1963 Apr 27;88(17):881-6.
10
A chemical investigation of the defects of myelination in phenylketonuria.苯丙酮尿症髓鞘形成缺陷的化学研究。
J Neurol Neurosurg Psychiatry. 1962 May;25(2):143-8. doi: 10.1136/jnnp.25.2.143.