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Stabilization by PRPP of Cellular Purine Phosphoribosyltransferases Against Inactivation by Freezing and Thawing. Study of Normal and Hypoxanthine-Guanine Phosphoribosyltransferase Deficient Human Fibroblasts

  • E. Zoref
  • O. Sperling
  • A. de Vries
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 41A)

Abstract

Hypoxanthine-guanine phosphoribosyltransferase (HGPRT, EC 2.4.2.8) and adenine phosphoribosyltransferase (APRT, EC 2.4.2.7) catalyze the salvage pathway formation of purine nucleotides from the corresponding preformed purine bases by reacting them with a common substrate, 5-phosphoribosyl-l-pyrophosphate (PRPP) (1,2). A deficiency of HGPRT in man causes excessive de novo production of purines. When the enzyme deficiency is virtually complete, it is associated with the Lesch-Nyhan syndrome (LNS) (3,4), when partial, it is associated with severe gout (5).

Keywords

Thermal Inactivation Human Skin Fibroblast Enzyme Deficiency Common Substrate Purine Nucleotide 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1974

Authors and Affiliations

  • E. Zoref
    • 1
  • O. Sperling
    • 1
  • A. de Vries
    • 1
    • 2
  1. 1.Tel-Aviv University Medical School, Department of Pathological ChemistrySheba Medical CenterTel-HashomerIsrael
  2. 2.Rogoff-Wellcome Medical Research InstituteBeilinson HospitalPetah TikvaIsrael

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