Fénéant M, Moatti N, Lemonnier F, Maccario J, Gautier M, Charpentier C, Lemonnier A
J Inherit Metab Dis. 1980;3(3):97-8. doi: 10.1007/BF02312539.
Glycine transport is studied in cultured skin fibroblasts from a patient with isolated hyperglycinuria and from five normal subjects. Fibroblasts from the patient take up glycine less well than do cell lines from controls. Kinetic studies are consistent with a single transport system in the patient's and controls' cell lines. Vmax value in the hyperglycinuric lines is normal, but the apparent affinity is always reduced as opposed to those of four different control lines separately tested. Statistical analysis shows significant difference between Km values.
对一名患有孤立性高甘氨酸尿症的患者以及五名正常受试者的培养皮肤成纤维细胞中的甘氨酸转运进行了研究。该患者的成纤维细胞摄取甘氨酸的能力不如对照组的细胞系。动力学研究表明,患者和对照组细胞系中存在单一转运系统。高甘氨酸尿症细胞系中的Vmax值正常,但与分别测试的四个不同对照组细胞系相比,其表观亲和力总是降低。统计分析表明Km值之间存在显著差异。