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亨廷顿舞蹈症患者的脑脊液和血清代谢谱:一项采用高分辨率质子核磁共振波谱法和高效液相色谱法的研究

CSF and serum metabolic profile of patients with Huntington's chorea: a study by high resolution proton NMR spectroscopy and HPLC.

作者信息

Nicoli F, Vion-Dury J, Maloteaux J M, Delwaide C, Confort-Gouny S, Sciaky M, Cozzone P J

机构信息

Centre de Résonance Magnétique Biologique et Médicale (CRMBM), URA CNRS 1186, Faculté de Médecine, Marseille, France.

出版信息

Neurosci Lett. 1993 May 14;154(1-2):47-51. doi: 10.1016/0304-3940(93)90168-k.

DOI:10.1016/0304-3940(93)90168-k
PMID:8361646
Abstract

We studied both cerebrospinal fluid (CSF) and serum of 11 patients suffering from Huntington's disease (HD) and 12 control subjects by combining high resolution proton NMR spectroscopy and HPLC. NMR spectroscopy analysis of the CSF shows a significant increase (60%) in pyruvate concentration in HD patients. No unexpected molecules were detected. Glutamate, glutamine, aspartate, proline and GABA levels were found unchanged in the CSF of HD patients, using HPLC analysis. Conversely, a significant increase (30%) in the CSF level of glycine was detected. These observations are in agreement with the metabolic hypothesis of HD physiopathogenesis. In addition, the protocol combining NMR spectroscopy and HPLC provides a straightforward evaluation of brain metabolic status and blood-brain-barrier function.

摘要

我们通过结合高分辨率质子核磁共振波谱法和高效液相色谱法,对11例亨廷顿舞蹈症(HD)患者及12名对照者的脑脊液(CSF)和血清进行了研究。脑脊液的核磁共振波谱分析显示,HD患者丙酮酸浓度显著升高(60%)。未检测到异常分子。采用高效液相色谱分析发现,HD患者脑脊液中的谷氨酸、谷氨酰胺、天冬氨酸、脯氨酸和γ-氨基丁酸水平未发生变化。相反,检测到脑脊液中甘氨酸水平显著升高(30%)。这些观察结果与HD生理病理发生的代谢假说相符。此外,结合核磁共振波谱法和高效液相色谱法的方案可直接评估脑代谢状态和血脑屏障功能。

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CSF and serum metabolic profile of patients with Huntington's chorea: a study by high resolution proton NMR spectroscopy and HPLC.亨廷顿舞蹈症患者的脑脊液和血清代谢谱:一项采用高分辨率质子核磁共振波谱法和高效液相色谱法的研究
Neurosci Lett. 1993 May 14;154(1-2):47-51. doi: 10.1016/0304-3940(93)90168-k.
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(1)H NMR based metabolomics of CSF and blood serum: a metabolic profile for a transgenic rat model of Huntington disease.基于氢核磁共振波谱的脑脊液和血清代谢组学:亨廷顿病转基因大鼠模型的代谢谱
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[Free aminoacids concentration in the plasma, red cell hemolysates and cerebrospinal fluid of patients with Huntington's chorea (author's transl)].
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[On changes in the composition of cerebrospinal fluid in the choreatic syndrome].[关于舞蹈病综合征中脑脊液成分的变化]
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What excitotoxin kills striatal neurons in Huntington's disease? Clues from neurochemical studies.在亨廷顿舞蹈症中,是哪种兴奋性毒素杀死了纹状体神经元?来自神经化学研究的线索。
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Human CSF GABA concentrations: revised downward for controls, but not decreased in Huntington's chorea.人类脑脊液中γ-氨基丁酸(GABA)浓度:对照组数据向下修正,但亨廷顿舞蹈症患者未降低。
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Huntington's disease - imbalance of free amino acids in the cerebrospinal fluid of patients and offspring at-risk.
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Plasma-aminoacid levels in Huntington's chorea.
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