Abstract
HPRT-deficient EB-virus transformed human lymphoblasts show decreased levels of ATP and GTP compared with control lymphoblasts. This decrease was not consistent.
The ATP content of the HPRT-deficient cells decreased to a significantly lower level than in control cells under unfavourable nutritional conditions, but not with frequent changes of medium. There was also a significant decrease in ADP-ribosylation in these cells.
The cells showed a significant positive correlation between ATP content and poly(ADP-ribose) synthetase activity and an inverse correlation between NAD content and poly(ADP-ribose) synthetase activity, as NAD is the substrate for this enzyme.
ADP-ribosylation is essential in many cellular processes including DNA repair and cell differentiation. So a decrease in ADP-ribosylation of nuclear protein may lead to decreased DNA repair. This may be a factor in explaining the long term deterioration of brain and testicular function in the Lesch-Nyhan Syndrome.
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Mccreanor, G. 92 REDUCED PURINE NUCLEOTIDE CONTENT AND POLY(ADP-RIBOSE) SYNTHETASE ACTIVITY IN HPRT-DEFICIENT HUMAN LYMPHOBLASTS. Pediatr Res 24, 126 (1988). https://doi.org/10.1203/00006450-198807000-00116
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DOI: https://doi.org/10.1203/00006450-198807000-00116