Article
GALT deficiency causes UDP-hexose deficit in human galactosemic cells.
Glycobiology - 1 Apr 2003
Lai K, Langley S D, Khwaja F W, Schmitt E W, Elsas L J
Abstract excerpt
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...
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