Regulation of de novo Purine Synthesis in the Lesch-Nyhan Syndrome

  • Gabrielle H. Reem
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 41A)


The deficiency of hypoxanthine guanine-phosphoribosyltransferase (HGPRT) activity of patients with the Lesch-Nyhan syndrome is accompanied by an accelerated rate of purine biosynthesis de novo. This elevated rate of purine biosynthesis is reflected in fibroblasts cultured from the skin of these patients and is accompanied by elevated levels of phosphoribosylpyrophosphate (PRPP).


Human Lymphocyte Purine Base Normal Lymphocyte Approximate Molecular Weight Purine Biosynthesis 
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Copyright information

© Plenum Press, New York 1974

Authors and Affiliations

  • Gabrielle H. Reem
    • 1
  1. 1.Department of PharmacologyNew York University Medical CenterUSA

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