Mar 1, 1976

The effect of pH on incorporation of galactose by a normal human cell line and cell lines from patients with defective galactose metabolism

Journal of Cellular Physiology
H Z Hill

Abstract

Incorporation of radioactive galactose into TCA-insoluble material of galactosemic fibroblasts is more sensitive to low pH than is the incorporation by normal human fibroblasts. This study was undertaken to determine (1) whether there was any pH which could correct or counteract the galactosemic defect relative to galactose incorporation, and (2) whether the low pH effect was specific for galactose metabolism or whether general cellular metabolism in galactosemic cells was more sensitive to low pH than that in normal cells. The pH dependencies of incorporation of radioactive galactose and glucose into cellular macromolecules were investigated in galactosemic and normal cells. Normal cells have a biphasic curve with respect to galactose incorporation with peaks at pH 7.0 and 8.5. Galactosemic cells have only the high pH peak. The maximum incorporation by galactosemic cells was never more than about 30% that seen by normal cells under the conditions of these experiments. Thus manipulation of the pH alone cannot correct the galactosemic defect. The rate of incorporation of radioactive galactose was studied in normal, galactosemic and galactokinase deficient cells, at pH 7.2 and at pH 6.3. At pH 7.2, galactosemic cells incorporate ...Continue Reading

Mentioned in this Paper

Metabolic Process, Cellular
Galactosemias
Biochemical Pathway
Galactokinase
Deficiency of Galactokinase
Galactose Measurement
Specimen Type - Fibroblasts
Thyroid Hormone Plasma Membrane Transport Defect
GALK1
Galactose

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