Iłzecka J, Stelmasiak Z, Solski J, Wawrzycki S, Szpetnar M
Department of Neurology, Medical University, Lublin, Poland.
Amino Acids. 2003 Jul;25(1):69-73. doi: 10.1007/s00726-002-0352-2.
Previous investigations showed an impairment of amino acids (AA) metabolism in amyotrophic lateral sclerosis (ALS). It was hypothesized that excitatory AA may play an important role in the etiopathogenesis of this disease. The aim of the study was to determine plasma AA concentrations in ALS patients, and to examine the relationship between AA and the clinical state of ALS patients, the type of ALS onset and the duration of the disease. The study involved 20 ALS patients and 30 control group people. The AA analysis was performed by ion - exchange chromatography on an automatic AA analyser. The results showed significantly decreased concentrations of valine, isoleucine, leucine, tyrosine and aspartate in the plasma of the whole group of ALS patients compared to the control group, and a significantly decreased concentration of arginine in the patients with a long duration of ALS compared to the patients with a short duration. The clinical state of ALS patients significantly influenced only plasma alanine concentration. Other plasma AA concentrations were not significantly associated with clinical parameters of the disease. Our study confirms that metabolic abnormalities concerning AA exist in ALS patients. However, the normal plasma glutamate concentration observed in this study in the whole group of ALS patients compared to the controls does not exclude that this excitatory AA may play a role in neurodegeneration in ALS.
先前的研究表明,肌萎缩侧索硬化症(ALS)患者存在氨基酸(AA)代谢障碍。据推测,兴奋性氨基酸可能在该疾病的病因发病机制中起重要作用。本研究的目的是测定ALS患者的血浆氨基酸浓度,并研究氨基酸与ALS患者临床状态、ALS发病类型及病程之间的关系。该研究纳入了20例ALS患者和30例对照组人员。采用离子交换色谱法在自动氨基酸分析仪上进行氨基酸分析。结果显示,与对照组相比,整个ALS患者组血浆中的缬氨酸、异亮氨酸、亮氨酸、酪氨酸和天冬氨酸浓度显著降低,与病程短的患者相比,病程长的ALS患者血浆中的精氨酸浓度显著降低。ALS患者的临床状态仅对血浆丙氨酸浓度有显著影响。其他血浆氨基酸浓度与该疾病的临床参数无显著相关性。我们的研究证实,ALS患者存在与氨基酸有关的代谢异常。然而,与对照组相比,本研究中整个ALS患者组血浆谷氨酸浓度正常,这并不排除这种兴奋性氨基酸可能在ALS神经退行性变中起作用。