GALT deficiency causes UDP-hexose deficit in human galactosemic cells.

@article{Lai2003GALTDC,
  title={GALT deficiency causes UDP-hexose deficit in human galactosemic cells.},
  author={Kent Lai and Sharon D Langley and Fatima W Khwaja and E William Schmitt and Louis J. Elsas},
  journal={Glycobiology},
  year={2003},
  volume={13 4},
  pages={285-94}
}
Previously we reported that stable transfection of human UDP-glucose pyrophosphorylase (hUGP2) rescued galactose-1-phosphate uridyltransferase (GALT)-deficient yeast from "galactose toxicity." Here we test in human cell lines the hypothesis that galactose toxicity was caused by excess accumulation of galactose-1-phosphate (Gal-1-P), inhibition of hUGP2, and UDP-hexose deficiency. We found that SV40-transformed fibroblasts derived from a galactosemic patient accumulated Gal-1-P from 1.2+/-0.4 to… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 33 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 42 references

Disorders of galactose metabolism

  • S. Segal, G. T. Berry
  • 1995
Highly Influential
8 Excerpts

Biogenesis of galactose, possible mechanism of self-intoxication in galactosemia

  • R. Gitzelmann, R. G. Hansen, B. Steinmann
  • 1984
Highly Influential
2 Excerpts

Similar Papers

Loading similar papers…