Patten B M, Harati Y, Acosta L, Jung S S, Felmus M T
Ann Neurol. 1978 Apr;3(4):305-9. doi: 10.1002/ana.410030405.
To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O-phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine. We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.
为评估肌萎缩侧索硬化症（ALS）患者游离氨基酸（AA）水平异常的报告，我们研究了12例ALS患者以及12例在年龄、性别和残疾严重程度方面相匹配的对照者的血清、脑脊液和尿液中的AA。ALS患者血清中的酪氨酸、总芳香族AA和总碱性AA水平有统计学意义的升高。ALS患者脑脊液中的总碱性AA、赖氨酸、必需AA和亮氨酸水平也有统计学意义的升高。ALS的严重程度与脑脊液中的酸性AA（谷氨酸和天冬氨酸）以及O - 磷酸丝氨酸呈负相关。ALS的活动与血清天冬氨酸和脑脊液丙氨酸呈正相关。我们得出结论，ALS患者存在AA水平的细微异常，且这些异常并非由年龄、性别或残疾所致。AA水平的分布模式不同于肝脏或肾脏疾病，提示中枢神经系统中碱性和必需AA的膜转运缺陷或细胞利用不良。
