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Control of pyruvate and beta-hydroxybutyrate utilization in rat brain mitochondria and its relevance to phenylketonuria and maple syrup urine disease.

作者信息

Land J M, Mowbray J, Clark J B

出版信息

J Neurochem. 1976 Apr;26(4):823-30. doi: 10.1111/j.1471-4159.1976.tb04458.x.

DOI:10.1111/j.1471-4159.1976.tb04458.x
PMID:987160
Abstract
摘要

相似文献

1
Control of pyruvate and beta-hydroxybutyrate utilization in rat brain mitochondria and its relevance to phenylketonuria and maple syrup urine disease.大鼠脑线粒体中丙酮酸和β-羟基丁酸利用的调控及其与苯丙酮尿症和枫糖尿症的相关性。
J Neurochem. 1976 Apr;26(4):823-30. doi: 10.1111/j.1471-4159.1976.tb04458.x.
2
[Phenylalanine and tyrosine in albino rat brain in the normal state and in experimental phenylketonuria].[正常状态及实验性苯丙酮尿症白化大鼠脑中的苯丙氨酸和酪氨酸]
Ukr Biokhim Zh. 1967;39(1):34-7.
3
Inhibition, by 2-oxo acids that accumulate in maple-syrup-urine disease, of lactate, pyruvate, and 3-hydroxybutyrate transport across the blood-brain barrier.枫糖尿症中积累的2-氧代酸对乳酸、丙酮酸和3-羟基丁酸跨血脑屏障转运的抑制作用。
J Neurochem. 1982 Sep;39(3):674-7. doi: 10.1111/j.1471-4159.1982.tb07945.x.
4
Transaminations between amino acids and keto acids elevated in phenylketonuria and maple syrup urine disease.苯丙酮尿症和枫糖尿症中氨基酸与酮酸之间的转氨作用升高。
J Neurochem. 1973 Feb;20(2):389-403. doi: 10.1111/j.1471-4159.1973.tb12138.x.
5
Inhibition of mitochondrial pyruvate transport by phenylpyruvate and alpha-ketoisocaproate.苯丙酮酸和α-酮异己酸对线粒体丙酮酸转运的抑制作用。
Biochim Biophys Acta. 1974 Oct 10;367(1):102-8. doi: 10.1016/0005-2736(74)90140-0.
6
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.
7
Possible biochemical model for phenylketonuria.苯丙酮尿症的可能生化模型。
Nature. 1972 Jan 28;235(5335):230. doi: 10.1038/235230a0.
8
Decreased essential amino acid requirements without catabolism in phenylketonuria and maple syrup urine disease.
Am J Clin Nutr. 1982 Feb;35(2):217-28. doi: 10.1093/ajcn/35.2.217.
9
Inhibition of pyruvate and beta-hydroxybutyrate oxidation in rat brain mitochondria by phenylpyruvate and alpha-ketoisocaproate.苯丙酮酸和α-酮异己酸对大鼠脑线粒体丙酮酸和β-羟基丁酸氧化的抑制作用。
FEBS Lett. 1974 Aug 30;44(3):348-51.
10
Large neutral amino acids auto exchange when infused by microdialysis into the rat brain: implication for maple syrup urine disease and phenylketonuria.通过微透析注入大鼠脑内时，中性大氨基酸会自动交换：对枫糖尿症和苯丙酮尿症的意义。
Neurochem Int. 2002 Apr;40(4):347-54. doi: 10.1016/s0197-0186(01)00077-8.

引用本文的文献

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Insights from Animal Models on the Pathophysiology of Hyperphenylalaninemia: Role of Mitochondrial Dysfunction, Oxidative Stress and Inflammation.动物模型对高苯丙氨酸血症病理生理学的研究进展：线粒体功能障碍、氧化应激和炎症的作用。
Mol Neurobiol. 2021 Jun;58(6):2897-2909. doi: 10.1007/s12035-021-02304-1. Epub 2021 Feb 6.
2
Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders.枫糖尿症中脑支链氨基酸：对神经紊乱的影响。
Int J Mol Sci. 2020 Oct 11;21(20):7490. doi: 10.3390/ijms21207490.
3
Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease.
枫糖尿症动物模型中急性给予支链氨基酸所诱导的凋亡信号通路。
Metab Brain Dis. 2017 Feb;32(1):115-122. doi: 10.1007/s11011-016-9892-0. Epub 2016 Aug 11.
4
Biochemical, Metabolic, and Behavioral Characteristics of Immature Chronic Hyperphenylalanemic Rats.未成熟慢性高苯丙氨酸血症大鼠的生化、代谢和行为特征
Neurochem Res. 2016 Feb;41(1-2):16-32. doi: 10.1007/s11064-015-1678-y. Epub 2015 Jul 30.
5
Acute Administration of Branched-Chain Amino Acids Increases the Pro-BDNF/Total-BDNF Ratio in the Rat Brain.急性给予支链氨基酸可增加大鼠脑中前体脑源性神经营养因子/总脑源性神经营养因子的比值。
Neurochem Res. 2015 May;40(5):885-93. doi: 10.1007/s11064-015-1541-1. Epub 2015 Feb 14.
6
Behavioral responses in rats submitted to chronic administration of branched-chain amino acids.长期给予支链氨基酸的大鼠的行为反应。
JIMD Rep. 2014;13:159-67. doi: 10.1007/8904_2013_274. Epub 2013 Nov 9.
7
Acute and chronic administration of the branched-chain amino acids decreases nerve growth factor in rat hippocampus.急性和慢性给予支链氨基酸可降低大鼠海马神经生长因子。
Mol Neurobiol. 2013 Dec;48(3):581-9. doi: 10.1007/s12035-013-8447-1. Epub 2013 Apr 5.
8
Chronic administration of branched-chain amino acids impairs spatial memory and increases brain-derived neurotrophic factor in a rat model.慢性支链氨基酸给药会损害空间记忆，并增加大鼠模型中的脑源性神经营养因子。
J Inherit Metab Dis. 2013 Sep;36(5):721-30. doi: 10.1007/s10545-012-9549-z. Epub 2012 Oct 30.
9
Evaluation of acetylcholinesterase in an animal model of maple syrup urine disease.评价枫糖尿症动物模型中的乙酰胆碱酯酶。
Mol Neurobiol. 2012 Apr;45(2):279-86. doi: 10.1007/s12035-012-8243-3. Epub 2012 Feb 12.
10
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.