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Science 28 September 1979:
Vol. 205. no. 4413, pp. 1395 - 1397
DOI: 10.1126/science.472754
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Articles

Science, Vol 205, Issue 4413, 1395-1397
Copyright © 1979 by American Association for the Advancement of Science

articles

Galactosemia: alterations in sulfate metabolism secondary to galactose-1-phosphate uridyltransferase deficiency

TA Tedesco and KL Miller

Cultures of nonmutant as well as galactokinase-deficient fibroblasts incorporate 20 percent more [35S]sulfate when galactose is substituted for glucose in the medium; galactose-1-phosphate uridyltransferase-deficient cells incorporate 65.5 percent less. In addition to incorporating less [35S]sulfate, the uridyltransferase-deficient cells showed significant accumulation of intracellular galactose-1-phosphate within 4 hours after galactose exposure. Under the same conditions, no difference in [3H]uridine incorporation was observed. This metabolic alteration, occurring in response to galactose exposure, may be related to the pathophysiology of classical galactosemia.


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Hum. Reprod. Update
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