Galactitol accumulation by glucose-6-phosphate deficient fibroblasts: a cellular model for resistance to the complications of diabetes mellitus.
When incubated in high galactose media, fibroblasts from individuals with the severe (Mediterranean) variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency accumulate significantly less galactitol than do fibroblasts from matched control subjects. The effect is not observed in fibroblasts from black subjects with the more common, and milder, A- variant of G6PD deficiency. Since aldose reductase and sorbitol dehydrogenase activities in experimental and control fibroblasts are identical, the effect is most likely due to the substantial reduction in NADPH levels in severely G6PD-deficient cells. Sorbitol does not accumulate either in control or in G6PD deficient fibroblasts incubated in high glucose medium, most likely because of the action of sorbitol dehydrogenase, and the presence of a carrier-mediated glucose transport system in the cell membrane which limits the concentration of glucose that can accumulate in these cells.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000449:Aldehyde Reductase / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D048909:Diabetes Complications; D005347:Fibroblasts / Q000378:metabolism*; D004376:Galactitol / Q000378:metabolism*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications / Q000378:metabolism*; D006801:Humans; D007064:L-Iditol 2-Dehydrogenase / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009249:NADP / Q000378:metabolism; D013012:Sorbitol / Q000378:metabolism; D013402:Sugar Alcohols / Q000378:metabolism*
D013402:Sugar Alcohols; D004376:Galactitol; D013012:Sorbitol; D009249:NADP; D007064:L-Iditol 2-Dehydrogenase; D000449:Aldehyde Reductase