Pourci M L, Mangeot M, Soni T, Lemonnier A
Laboratoire de Biochimie Appliquée, Faculté de Pharmacie, Châtenay-Malabry, Université Paris-Sud, France.
J Inherit Metab Dis. 1990;13(6):819-28. doi: 10.1007/BF01800205.
Fibroblasts from three galactosaemics had no galactose-1-phosphate uridyltransferase (GALT) activity. These fibroblasts cells were cultured in different media supplemented with dialysed fetal calf serum. Galactosaemic and control cell strains stopped growing in hexose-free medium. In glucose-free medium containing galactose, galatosaemic cells, in contrast to control cells, stopped growing after two days and died. In the same medium supplemented with inosine, they exhibited the same growth pattern as the control cell strains although in the presence of high concentrations of galactose-1-phosphate (Gal-1-P). These findings indicated that the glucose-free medium containing galactose supplemented with dialysed fetal calf serum and inosine, as a ribose donor, was appropriate for further in vitro investigations of galactose metabolism in galactosaemic cells.
三名半乳糖血症患者的成纤维细胞没有1-磷酸半乳糖尿苷转移酶（GALT）活性。这些成纤维细胞在添加了透析胎牛血清的不同培养基中培养。半乳糖血症细胞株和对照细胞株在无己糖培养基中停止生长。在含有半乳糖的无葡萄糖培养基中，与对照细胞相比，半乳糖血症细胞在两天后停止生长并死亡。在添加了肌苷的相同培养基中，尽管存在高浓度的1-磷酸半乳糖（Gal-1-P），它们仍表现出与对照细胞株相同的生长模式。这些发现表明，含有半乳糖、添加了透析胎牛血清和作为核糖供体的肌苷的无葡萄糖培养基，适用于对半乳糖血症细胞中半乳糖代谢进行进一步的体外研究。
