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Phenylketonuria: metabolic alterations induced by phenylalanine and phenylpyruvate.

作者信息

Patel M S, Arinze I J

出版信息

Am J Clin Nutr. 1975 Feb;28(2):183-8. doi: 10.1093/ajcn/28.2.183.

DOI:10.1093/ajcn/28.2.183
PMID:234671
Abstract
摘要

相似文献

1
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.
2
Pyruvate metabolism by homogenates of human brain: effects of phenylpyruvate and implications for the etiology of the mental retardation in phenylketonuria.人脑匀浆中丙酮酸的代谢:苯丙酮酸的影响及对苯丙酮尿症智力发育迟缓病因的意义
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3
[Study on experimental phenylketonuria].
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Glutamine depletion in phenylketonuria. A possible cause of the mental defect.
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Two siblings of hyperphenylalaninemia: suggestion to a genetic variant of phenylketonuria.
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Phenylketonemia in phenylketonuria.
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Cerebral lipid metabolism in experimental hyperphenylalaninaemia: incorporation of 14C-labelled glucose into total lipids.
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One too many: intellectual disability secondary to undiagnosed phenylketonuria.过量之害:未确诊苯丙酮尿症继发的智力残疾。
Hong Kong Med J. 2016 Oct;22(5):506-8. doi: 10.12809/hkmj144500.
9
Biochemical effects of induced phenylketonuria in rats.
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10
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.

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Airway microbiota associated D-phenylalanine promotes non-small cell lung cancer metastasis through epithelial mesenchymal transition.气道微生物群相关的D-苯丙氨酸通过上皮-间质转化促进非小细胞肺癌转移。
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Glutamine energy substrate anaplerosis increases bone density in the Pah classical PKU mouse in the absence of phenylalanine restriction.在不存在苯丙氨酸限制的情况下,谷氨酰胺能量底物回补增加了苯丙酮尿症经典型苯丙酮尿症小鼠的骨密度。
JIMD Rep. 2022 Jul 6;63(5):446-452. doi: 10.1002/jmd2.12308. eCollection 2022 Sep.
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Phenylketonuria oxidative stress and energy dysregulation: Emerging pathophysiological elements provide interventional opportunity.
苯丙酮尿症氧化应激和能量失调:新兴的病理生理因素提供了干预机会。
Mol Genet Metab. 2022 Jun;136(2):111-117. doi: 10.1016/j.ymgme.2022.03.012. Epub 2022 Mar 29.
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Comparative metabolomics in the Pah classical PKU mouse identifies cerebral energy pathway disruption and oxidative stress.在 Pah 经典 PKU 小鼠中的比较代谢组学研究确定了大脑能量途径的破坏和氧化应激。
Mol Genet Metab. 2022 May;136(1):38-45. doi: 10.1016/j.ymgme.2022.03.004. Epub 2022 Mar 18.
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Mesenchymal stem cell energy deficit and oxidative stress contribute to osteopenia in the Pah classical PKU mouse.间充质干细胞能量不足和氧化应激导致 Pah 经典型 PKU 小鼠的骨质疏松症。
Mol Genet Metab. 2021 Mar;132(3):173-179. doi: 10.1016/j.ymgme.2021.01.014. Epub 2021 Feb 11.
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Plasma and skeletal muscle amino acids following severe burn injury in patients and experimental animals.严重烧伤患者和实验动物伤后的血浆及骨骼肌氨基酸情况
Ann Surg. 1982 Jan;195(1):75-89. doi: 10.1097/00000658-198201001-00012.
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Effect of hyperphenylalaninaemia on lipid synthesis from ketone bodies by rat brain.高苯丙氨酸血症对大鼠脑利用酮体合成脂质的影响。
Biochem J. 1976 Feb 15;154(2):319-25. doi: 10.1042/bj1540319.