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Science 12 December 1975:
Vol. 190. no. 4219, pp. 1098 - 1099
DOI: 10.1126/science.171772
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Articles

Science, Vol 190, Issue 4219, 1098-1099
Copyright © 1975 by American Association for the Advancement of Science

articles

Phosphoribosylpyrophosphate overproduction, a new metabolic abnormality in the Lesch Nyhan syndrome

GH Reem

The activity of phosphoribosylpyrophosphate synthetase and the rate of phosphoribosylpyrophosphate accumulation are significantly increased in cultured lymphocytes of Lesch Nyhan patients deficient in hypoxanthine guanine phosphoribosyltransferase and in a clone of mutagen-induced, hypoxanthine guanine phosphoribosyltransferase deficient human lymphocytes. The increase in phosphoribosylpyrophosphate synthetase activity is the cause of the abnormally high cellular phosphoribosylpyrophosphate content and possibly of the purine overproduction described in this syndrome.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Phosphoribosylpyrophosphate synthesis in cultured human cells.
P. Benke and D Dittmar (1977)
Science 198, 1171-1173
   Abstract »    PDF »
Phosphoribosylpyrophosphate synthetase is elevated in fibroblasts from patients with the Lesch-Nyhan syndrome.
D. Martin Jr and B. Maler (1976)
Science 193, 408-411
   Abstract »    PDF »


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