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患有一种高度流行的运动神经元疾病——骨软化病患者脑脊液中的抑制性和兴奋性氨基酸。

Inhibitory and excitatory amino acids in cerebrospinal fluid of neurolathyrism patients, a highly prevalent motorneurone disease.

作者信息

Khan J K, Kuo Y H, Haque A, Lambein F

机构信息

Laboratory of Physiological Chemistry, University of Ghent, Belgium.

出版信息

Acta Neurol Scand. 1995 Jun;91(6):506-10. doi: 10.1111/j.1600-0404.1995.tb00454.x.

Abstract

Neurolathyrism is caused by overconsumption of seeds containing 3-N-oxalyl-L-2,3-diaminopropanoic acid (beta-ODAP). Amino acids levels of cerebrospinal fluid (CSF) were studied in 50 patients with neurolathyrism and 12 healthy volunteers. The levels of excitatory amino acids glutamate and aspartate were 281% and 71% respectively of control values. The concentration of inhibitory amino acids glycine and taurine were 277% and 198% respectively of the levels in CSF from control individuals. There was a significant correlation between the level of glycine and the duration of the disease. We also found increased levels of threonine, serine and alanine. In contrast to reports on other motor neurone diseases where an increase of isoleucine was observed we found a significant decrease of isoleucine. The results suggest a disturbance of amino acid metabolism due to excitotoxic damages caused by beta-ODAP, a dietary excitatory amino acid.

摘要

神经病性骨软化症是由于过度食用含有3 - N - 草酰 - L - 2,3 - 二氨基丙酸(β - ODAP)的种子所致。对50例神经病性骨软化症患者和12名健康志愿者的脑脊液(CSF)氨基酸水平进行了研究。兴奋性氨基酸谷氨酸和天冬氨酸的水平分别是对照值的281%和71%。抑制性氨基酸甘氨酸和牛磺酸的浓度分别是对照个体脑脊液中水平的277%和198%。甘氨酸水平与疾病持续时间之间存在显著相关性。我们还发现苏氨酸、丝氨酸和丙氨酸水平升高。与其他观察到异亮氨酸增加的运动神经元疾病报告相反,我们发现异亮氨酸显著减少。结果表明,由于饮食中的兴奋性氨基酸β - ODAP引起的兴奋性毒性损伤,导致了氨基酸代谢紊乱。

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