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Urea Cycle Diseases

  • Book
  • © 1982

Overview

Editors:
  1. A. Lowenthal

    1. Born-Bunge Foundation, Universitaire Instelling Antwerpen, Wilrijk, Belgium

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  2. A. Mori

    1. Institute for Neurobiology, Okayama University Medical School, Okayama, Japan

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  3. B. Marescau

    1. Born-Bunge Foundation, Universitaire Instelling Antwerpen, Wilrijk, Belgium

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Part of the book series: Advances in Experimental Medicine and Biology (AEMB, volume 153)

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Table of contents (62 chapters)

  1. Diagnostic, Clinical, Pathological and Biochemical Aspects of the Diseases

    1. Secondary and Transient Hyperammonemia

      1. Hyperammonemia Secondary to Hereditary Organic Acidurias : A Study of 29 Cases
        • J. M. Saudubray, F. X. Coudé, H. Ogier, L. Cathelineau, P. Briand, C. Charpentier
        Pages 135-140
      2. Neonatal Isovaleric Acidemia Associated with Hyperammonemia
        • M. Yoshino, I. Yoshida, F. Yamashita, M. Mori, C. Uchiyama, M. Tatibana
        Pages 141-146
      3. Hyperammonemia in the Neonate with Hypoxia
        • Yusuke Sakaguchi, Ken Yuge, Makoto Yoshino, Fumio Yamashita, Takeo Hashimoto
        Pages 147-152
      4. A Mechanism for Valproate-Induced Hyperammonemia
        • F. X. Coudé, D. Rabier, L. Cathelineau, G. Grimber, P. Parvy, P. Kamoun
        Pages 153-161
      5. Reduced Activity of OTC in the Liver of a Patient with Reye’s Syndrome
        • Shinichiro Arashima, Yasuro Takekoshi, Mishiya Anakura Anakura, Haruo Nanbu, Ichiro Matsuda
        Pages 163-170

  2. Basic Biochemistry

    1. Front Matter

      Pages 195-195
      Download chapter PDF

    2. Regulation

      1. Regulation of the N-Acetylglutamate Content of Rat Hepatocytes by the Glutamate Concentration
        • H. Zollner
        Pages 197-205
      2. Enzyme Regulation of <u>N</u>-Acetylglutamate Synthesis in Mouse and Rat Liver
        • Masamiti Tatibana, Susumu Kawamoto, Tomoko Sonoda, Masataka Mori
        Pages 207-216
      3. Acute Glucagon Treatment in Rats Fed Various Protein Diets Effect on N-Acetyl Glutamate Concentration
        • L. Cathelineau, D. Rabier, F. X. Coudé
        Pages 217-228
      4. The Relation Between the Developmental Timing of Birth and Developmental Increases in Urea Cycle Enzymes
        • W. H. Lamers, P. G. Mooren, W. Oosterhuis, H. Lunstroo, A. De Graaf, R. Charles
        Pages 229-240
      5. Studies on the Enzymes of Urea Cycle Intermediates in Normal and Infarcted Myocardial Tissue of Rat
        • B. Sadasivudu, M. Swamy, G. N. Rao
        Pages 241-241
      6. Effects of Arginine-Free Meals on Ureagenesis in Cats
        • P. M. Stewart, M. Batshaw, D. Valle, M. Walser
        Pages 243-243
      7. Dynamism of Rat Liver Ornithine Metabolisms in Relation to Dietary High-Protein Stimulation of the Urea Cycle
        • Takeo Matsuzawa, Naofumi Sugimoto, Isao Ishiguro
        Pages 245-254
      8. Regulation of Urea Synthesis : Changes in the Concentration of Ornithine in the Liver Corresponding to Changes in Urea Synthesis
        • T. Saheki, M. Hosoya, S. Fujinami, T. Katsunuma
        Pages 255-263

    3. Enzymes

      1. Synthesis and Intracellular Transport of Mitochondrial Carbamyl Phosphate Synthetase I and Ornithine Transcarbamylase
        • Masataka Mori, Satoshi Miura, Tetsuo Morita, Masamiti Tatibana
        Pages 267-276
      2. Isolation of Argininosuccinase from Bovine Brain : Catalytic, Physical and Chemical Properties Compared to Liver and Kidney Enzymes
        • Kimiko Murakami-Murofushi, Sarah Ratner
        Pages 277-289
      3. On the Mechanism of the Alterations of Rat Kidney Transamidinase Activities by Diet and Hormones
        • John F. Van Pilsum, Denise M. McGuire, Howard Towle
        Pages 291-297
      4. Sciatectomic Stimulation of Muscle Arginase and Its Implications
        • V. Mohanachari, P. Neeraja, K. Indira, K. S. Swami
        Pages 299-301
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Keywords

About this book

Introduction New Facets in Urea Cycle Disorders INTRODUCTION A. Lowenthal Laboratory of Neurochemistry, Born-Bunge Foundation, Universitaire Instelling Antwerpen, Wilrijk, Belgium This occasion is by no means the first meeting devoted to urea cycle diseases. It has been preceeded by meetings held in the Netherlands and in Spain. Accordingly the justification for a further meeting is not immediately evident. The reason for it is that the problems related to urea cycle diseases are developing fast, as instanced inter alia by I) the relation observed between hyperornithinemia and gyrate atrophy and the therapeutic acquisitions which result from it 2) the treatment of hyperammonemias. If the diagnosis of urea cycle disease is easily established by following standard principles and techniques, i.e. by means of amino acid analysis, with or without prior loading tests, by ammonemia measurement and by enzyme determination, also if these operations produce clear and precise conclusions in the matter of genetics and preventive medicine, yet many physiopathological questions remain unanswered and a number of therapeutic problems of these remain unsolved.

Editors and Affiliations

  • Born-Bunge Foundation, Universitaire Instelling Antwerpen, Wilrijk, Belgium

    A. Lowenthal, B. Marescau

  • Institute for Neurobiology, Okayama University Medical School, Okayama, Japan

    A. Mori

Bibliographic Information

  • Book Title: Urea Cycle Diseases

  • Editors: A. Lowenthal, A. Mori, B. Marescau

  • Series Title: Advances in Experimental Medicine and Biology

  • DOI: https://doi.org/10.1007/978-1-4757-6903-6

  • Publisher: Springer New York, NY

  • eBook Packages: Springer Book Archive

  • Copyright Information: The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature 1982

  • Softcover ISBN: 978-1-4757-6905-0Published: 03 March 2013

  • eBook ISBN: 978-1-4757-6903-6Published: 09 March 2013

  • Series ISSN: 0065-2598

  • Series E-ISSN: 2214-8019

  • Edition Number: 1

  • Number of Pages: XV, 528

  • Topics: Biochemistry, general

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