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Galactose and glucose metabolism in galactokinase deficient, galactose-1-P-uridyl transferase deficient and normal human fibroblasts.
J Cell Physiol. 1975 Jun; 85(3):569-78.JC

Abstract

Despite the genetic interruption of the Leloir pathway both galactosemic patients and galactosemic fibroblasts can convert galactose to CO2 and TCA precipitable products, although at less than the normal rate. These observations stimulated investigations into the identity of the alternative metabolic routes which allows for galactose metabolism in the absence of in vitro galactose-1-P-uridyl transferase. Four lines of galactosemic cells, each without detectable gal-transferase, produced 14CO2 from [1-14C]-galactose (0.094 mumoles in 20 cc of medium) at approximately 39% +/- 16% the rate of transferase positive cells over a 48-hour period. However, galactokinase deficient fibroblasts produced 14CO2 and TCA precipitable products from [1-14C]-galactose or [U-14C]-galactose at only 3% to 9% the rate of normal fibroblasts. Therefore it seems likely that gal-transferase deficient fibroblasts must first synthesize galactose-1-P for further metabolism of galactose.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

167035

Citation

Friedman, T B., et al. "Galactose and Glucose Metabolism in Galactokinase Deficient, galactose-1-P-uridyl Transferase Deficient and Normal Human Fibroblasts." Journal of Cellular Physiology, vol. 85, no. 3, 1975, pp. 569-78.
Friedman TB, Yarkin RJ, Merril CR. Galactose and glucose metabolism in galactokinase deficient, galactose-1-P-uridyl transferase deficient and normal human fibroblasts. J Cell Physiol. 1975;85(3):569-78.
Friedman, T. B., Yarkin, R. J., & Merril, C. R. (1975). Galactose and glucose metabolism in galactokinase deficient, galactose-1-P-uridyl transferase deficient and normal human fibroblasts. Journal of Cellular Physiology, 85(3), 569-78.
Friedman TB, Yarkin RJ, Merril CR. Galactose and Glucose Metabolism in Galactokinase Deficient, galactose-1-P-uridyl Transferase Deficient and Normal Human Fibroblasts. J Cell Physiol. 1975;85(3):569-78. PubMed PMID: 167035.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Galactose and glucose metabolism in galactokinase deficient, galactose-1-P-uridyl transferase deficient and normal human fibroblasts. AU - Friedman,T B, AU - Yarkin,R J, AU - Merril,C R, PY - 1975/6/1/pubmed PY - 1975/6/1/medline PY - 1975/6/1/entrez SP - 569 EP - 78 JF - Journal of cellular physiology JO - J Cell Physiol VL - 85 IS - 3 N2 - Despite the genetic interruption of the Leloir pathway both galactosemic patients and galactosemic fibroblasts can convert galactose to CO2 and TCA precipitable products, although at less than the normal rate. These observations stimulated investigations into the identity of the alternative metabolic routes which allows for galactose metabolism in the absence of in vitro galactose-1-P-uridyl transferase. Four lines of galactosemic cells, each without detectable gal-transferase, produced 14CO2 from [1-14C]-galactose (0.094 mumoles in 20 cc of medium) at approximately 39% +/- 16% the rate of transferase positive cells over a 48-hour period. However, galactokinase deficient fibroblasts produced 14CO2 and TCA precipitable products from [1-14C]-galactose or [U-14C]-galactose at only 3% to 9% the rate of normal fibroblasts. Therefore it seems likely that gal-transferase deficient fibroblasts must first synthesize galactose-1-P for further metabolism of galactose. SN - 0021-9541 UR - https://www.unboundmedicine.com/medline/citation/167035/Galactose_and_glucose_metabolism_in_galactokinase_deficient_galactose_1_P_uridyl_transferase_deficient_and_normal_human_fibroblasts_ L2 - https://doi.org/10.1002/jcp.1040850308 DB - PRIME DP - Unbound Medicine ER -