Summary
Fibroblasts from three galactosaemics had no galactose-1-phosphate uridyltransferase (GALT) activity. These fibroblast 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 furtherin vitro investigations of galactose metabolism in galactosaemic cells.
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Pourci, M.L., Mangeot, M., Soni, T. et al. Culture of galactosaemic fibroblasts in the presence of galactose: Effect of inosine. J Inherit Metab Dis 13, 819–828 (1990). https://doi.org/10.1007/BF01800205
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DOI: https://doi.org/10.1007/BF01800205