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Purine Metabolism in Man—II

Regulation of Pathways and Enzyme Defects

  • Mathias M. Müller
  • Erich Kaiser
  • J. Edwin Seegmiller

Table of contents

  1. Front Matter
    Pages i-xxii
  2. History of Gout

    1. Andre de Vries
      Pages 1-12
  3. Metabolic Pathways of Purines

    1. De Novo Synthesis: Precursors and Regulation

      1. Peter B. Rowe, Gemma Madsen, E. McCairns, Dorit Sauer
        Pages 13-18
      2. Floyd F. Snyder, Michael S. Hershfield, J. Edwin Seegmiller
        Pages 30-39
    2. De Novo Synthesis: Phosphoribosylpyrophosphate and Phosphoribosylpyrophosphate Synthetase

    3. De Novo Synthesis: Phosphoribosylpyrophosphate Amidotransferase

    4. Nucleotide Metabolism

      1. Merrie M. Johnson, David Nash, J. Frank Henderson
        Pages 110-114
      2. Donald J. Nelson, Christoper Buggé, Harvey C. Krasny
        Pages 121-128
    5. Salvage Pathway

      1. C. H. M. M. de Bruyn, T. L. Oei
        Pages 139-152
      2. Vassilis I. Zannis, Lorraine J. Gudas, Deborah Doyle, David W. Martin Jr.
        Pages 162-171
      3. C. H. M. M. de Bruyn, T. L. Oei, M. P. Uitendaal, P. Hösli
        Pages 172-180
      4. Gabrielle H. Reem, Charlotte Friend
        Pages 181-185
    6. Catabolism

      1. J. S. Cameron, H. A. Simmonds, A. Cadenhead, D. Farebrother
        Pages 196-205
      2. Nobuaki Ogasawara, Haruko Goto, Tomomasa Watanabe
        Pages 212-222
      3. P. E. Daddona, W. N. Kelley
        Pages 223-234
      4. Irving H. Fox, P. Marchant
        Pages 249-253
  4. Mutations Affecting Purine Metabolism in Man

    1. General Aspects

    2. Phosphoribosylpyrophosphate Synthetase

    3. Adenine Phosphoribosyltransferase

      1. B. T. Emmerson, L. A. Johnson, R. B. Gordon
        Pages 293-294
      2. K. J. Van Acker, H. A. Simmonds, J. S. Cameron
        Pages 295-302
    4. Hypoxanthine-Guanine Phosphoribosyltransferase : Partial Deficiency

    5. Hypoxanthine-Guanine Phosphoribosyltransferase : Complete Deficiency (Lesch-Nyhan Syndrome)

About this book

Introduction

The study of gouty arthritis has provided a common meeting ground for the research interests of both the basic scientist and the clinician. The interest of the chemist in gout began 1776 with the isolation of uric acid from a concretion of the urinary tract by the Swedish chemist SCHEELE. The same substance was subsequently extracted from a gouty tophus by the British chemist WOLLASTONE in 1797 and a half century later the cause of the deposits of sodium urate in such tophi was traced to a hyperuricemia in the serum of gouty patients by the British physician Alfred Baring GARROD who had also received training in the chemical laboratory and was therefore a fore-runner of many of today's clinician-investigators. The recent surge of progress in understanding of some of the causes of gout in terms of specific enzyme defects marks the entrance of the biochemist into this field of investigation. The identification of the first primary defect of purine metabolism associated with over-production of uric acid, a severe or partial deficiency of the enzyme hypoxanthine-guanine phospho­ ribosyltransferase was achieved less than a decade ago. The knowledge of the mechanism of purine over-production that it generated led shortly to the identification of families carrying a dominantly (possibly X-linked) inherited increase in the activity of the enzyme phosphoribosylpyrophosphate synthetase as a cause of purine over-production. Yet this is only a start as these two types of enzyme defects account for less than five per cent of gouty patients.

Keywords

Fructose Glutamin Lipid NADP Nucleotide Purine Pyrimidine carbohydrates enzyme enzymes metabolism mutation pharmacology protein synthesis

Editors and affiliations

  • Mathias M. Müller
    • 1
  • Erich Kaiser
    • 1
  • J. Edwin Seegmiller
    • 2
  1. 1.University of ViennaViennaAustria
  2. 2.University of California, San DiegoLa JollaUSA

Bibliographic information