Abstract
The utilization of fructose and glucose by fibroblast cultures obtained from patients with hereditary fructose intolerance (HFI) was studied in comparison with fibroblast controls. The cell growth, the time course ofd-glucose ord-fructose uptake and the consumption of fructose were similar for both HFI and control cells. Some results showed significant differences between these two cell types: HFI cells consumed less glucose, produced less lactate and contained less glycogen than control cells. Furthermore, significantly less [U-14C]d-glucose and [U-14C]d-fructose was incorporated into lipids in HFI cells than in control cells. The mechanisms responsible for these differences observed between the two cell types are not known.
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References
Bligh, E. and Dyer, W. A rapid method of total lipid extraction and purification.J. Biochem. Physiol. 37 (1952) 911–917
Burton, B. K., Chacko, C. M. and Nadler, H. L. Aldolase in cultivated human fibroblasts.Proc. Soc. Exp. Biol. Med. 146 (1974) 605–607
Delhotal, B., Lemonnier, F., Couturier, M., Wolfrom, C., Gautier, M. and Lemonnier, A. Comparative use of fructose and glucose in human liver and fibroblastic cell cultures.In Vitro 20 (1984) 699–706
Gliksman, R., Ghosh, N. K. and Cox, R. P. Comparison of aldolase isozymes in placenta, HeLa cells, and human fibroblast cultures.Enzyme 22 (1977) 416–419
Gregori, C., Schapira, F., Kahn, A., Delpech, M. and Dreyfus, J. C. Molecular studies of liver aldolase B in hereditary fructose intolerance using blotting and immunological techniques.Ann. Hum. Genet. 46 (1982) 281–292
Hers, H. G. and Joassin, G. Anomalies de l'aldolase hépatique dans l'intolerance au fructose.Enzymol. Biol. Clin. 1 (1961) 4–14
Imamura, T., Crespi, C. L., Thilly, W. G. and Brunengraber, H. Fructose as a carbohydrate source yields stable pH and redox parameters in microcarrier cell culture.Anal. Biochem. 124 (1982) 353–358
Johnson, G. S. and Schwartz, J. P. Effects of sugars on the physiology of cultured fibroblasts.Exp. Cell. Res. 97 (1976) 281–290
Lemonnier, F., Gautier, M., Moatti, N. and Lemonnier, A. Comparative study of extracellular aminoacids in culture of human liver and fibroblastic cells.In Vitro 12 (1976) 460–466
Lowry, O., Rosebrough, N., Farr, A. and Randall, R. Protein measurement with the folin phenol reagent.J. Biol. Chem. 193 (1951) 265–275
Nahorski, S. R. and Rogers, K. J. An enzymic fluorometric micro method for determination of glycogen.Anal. Biochem. 49 (1972) 492–497
Odièvre, M., Gentil, C., Gautier, M. and Alagille, D. Hereditary fructose intolerance in childhood. Diagnosis, management and course in 55 patients.Am. J. Dis. Child. 132 (1978) 605–608
Reitzer, L. J., Wice, B. M. and Kennell, D. Evidence that glutamine, not sugar, is the major energy source for cultured HeLa cells.J. Biol. Chem. 8 (1979) 2669–2676
Schapira, F. L'activité fructose-1-phosphoaldolasique des tissus de mammifères.Bull. Soc. Chim. Biol. 43 (1961) 1357–1387
Shin, Y. S., Moro, V., Doliwa, H. and Endres, W. A radioisotopic method for fructose-1-phosphate aldolase assay that facilitates diagnosis of hereditary fructose intolerance.Clin. Chem. 29 (1983) 1955–1958
Vessal, M., Choun, M. O., Bissell, M. J. and Bissell, D. Fructose utilization and altered cytochrome P-450 in cultured hepatocytes from adult rats.Biochim. Biophys. Acta 633 (1980) 201–210
Vrana, A. and Fabry, P. Nutrients and Energy. In Bourne, G. (ed.)World Review of Nutrition and Dietetics, Vol. 42, Karger, Basel, 1983 pp. 56–101
Wolfrom, C., Loriette, C., Polini, G., Delhotal, B., Lemonnier, F. and Gautier, M. Comparative effects of glucose and fructose in growth and morphological aspects of cultured skin fibroblasts.Exp. Cell. Res. 149 (1983) 535–546
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Lemonnier, F., Delhotal-Landes, B., Couturier, M. et al. Comparative use of glucose and fructose in cultured fibroblasts from patients with hereditary fructose intolerance. J Inherit Metab Dis 10, 52–61 (1987). https://doi.org/10.1007/BF01799488
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DOI: https://doi.org/10.1007/BF01799488