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Lipid Storage Disorders

Biological and Medical Aspects

  • Conference proceedings
  • © 1988

Overview

Editors:
  1. Robert Salvayre

    1. Paul Sabatier University, Toulouse, France

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  2. Louis Douste-Blazy

    1. Paul Sabatier University, Toulouse, France

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  3. Shimon Gatt

    1. Hebrew University—Hadassah School of Medicine, Jerusalem, Israel

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Part of the book series: NATO Science Series A: (NSSA, volume 150)

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Table of contents (102 papers)

  1. Front Matter

    Pages i-xvii
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  2. Lysosomal Storage Diseases

    1. Front Matter

      Pages 1-1
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    2. β-glucosidases and Gaucher diseases

      1. The Medical Importance of the Research on Lipid Storage Diseases with a Historical Review on the Advances in Gaucher Disease
        • Lars Svennerholm
        Pages 5-17
      2. The Molecular Biology of Gaucher Disease
        • E. Beutler, J. Sorge, A. Zimran, C. West, W. Kuhl, B. Westwood et al.
        Pages 19-27
      3. Characterization of the Normal Human Glucocerebrosidase Genes and a Mutated Form in Gaucher’s Patient
        • Orly Reiner, Meir Wigderson, Mia Horowitz
        Pages 29-39
      4. Molecular Biology of Gaucher Disease: Therapeutic Strategies Utilizing Recombinant DNA Technology
        • Brian M. Martin, Shoji Tsuji, Mary E. LaMarca, Kara Maysak, William Eliason, Edward I. Ginns
        Pages 41-50
      5. Molecular Properties of Lysosomal Glucocerebrosidase
        • S. Van Weely, J. M. F. G. Aerts, M. Van Leeuwen, M. E. Peterson, J. Goldblatt, J. M. Tager et al.
        Pages 51-56
      6. Comparison of Human Membrane-Bound β-Glucosidases: Lysosomal Glucosylceramide-β-Glucosidase and Non-Specific β-Glucosidase
        • A. Maret, R. Salvayre, M. Potier, G. Legler, G. Beauregard, L. Douste-Blazy
        Pages 57-61
      7. β-Glucocerebrosidase: Mechanistic Studies With Covalent and Non-Covalent Inhibitors
        • GĂĽnter Legler
        Pages 63-72
      8. Comparison of the Acidic Lipid Requirement of Control and Type 1 Gaucher’s Disease Liver and Brain Glucocerebrosidases
        • Barbara A. Butcher, Robert E. Lee, Alakananda Basu, Robert H. Glew
        Pages 73-82
      9. Heterogeneity in Human Acid β-Glucosidase with Cellulose-Acetate Electrophoresis
        • M. C. Sa Miranda, R. Pinto, A. W. Schram, J. M. Tager, J. M. F. G. Aerts, J. A. Barranger
        Pages 83-88
      10. Etiology of a New Identified Gaucher Disease Variant without Glucosylceramidase Defect
        • Helen Christomanou, Annemarie Aignesberger, Norbert Herschkowitz, Ulrich N. Wiesmann
        Pages 89-95
      11. Type 3 Gaucher Disease: Clinical and Biological Heterogeneity
        • I. Maire, P. Guibaud
        Pages 97-101
      12. Parkinsonian Symptomatology in a Patient with Type I (Adult) Gaucher’s Disease
        • J. C. Turpin, G. Dubois, A. Brice, M. Masson, M. C. Nadaud, J. M. Boutry et al.
        Pages 103-105

    3. Sphingomyelinase and Niemann-Pick disease types A and B

      1. Acid Sphingomyelinase from Human Urine: Purification and Characterisation
        • L. E. Quintern, H. Nehrkorn, K. Sandhoff, G. Weitz, J. M. Tager, A. W. Schram
        Pages 109-118
      2. Lysosomal Sphingomyelinase: Patients with Niemann-Pick Disease Have Normal Amounts of Sphingomyelinase Polypeptide
        • J. W. Callahan, E. A. Jobb
        Pages 119-128
      3. Sphingomyelin Storage in Lymphoid Cell Lines from Patients with Niemann-Pick Disease Types A, B and C: Influence of Culture Conditions
        • Thierry Levade, Robert Salvayre, Arlette Maret, Louis Douste-Blazy
        Pages 129-133
      4. Turnover of Docosahexaenoic Acid in Bis(Monoacylglycero)Phosphate Induced in Niemann-Pick Fibroblasts by Incubation in the Presence of Excess Fatty Acid
        • Srebrenka Huterer, John Wherrett
        Pages 135-140
      5. Heterogeneity and Special Features of the Storage Process in Niemann-Pick Disease
        • Milan Elleder
        Pages 141-151
      6. Family with Profound Sphingomyelinase Deficiency Resisting Closer Subclassification
        • Milan Elleder, Jiri Cihula, Jaromir Sverak, Marie-ThĂ©rèse Vanier
        Pages 153-161
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Keywords

About this book

This book presents the proceedings of the meeting on "Lipid Storage Disorders" which took place in Toulouse, France, in September 14-18, 1987 and which was set up as a joint NATO Advanced Research Workshop and INSERM International Symposium. The meeting probably was the first truly international symposium devoted entirely to basic as well as applied aspects of lipid metabolism as related to the lipid storage disorders. Participants came from Europe, USA, Israel and Japan, and presented data on research in molecular biology and genetics, enzymology, cell biology as well as medical and epidemiological aspects of normal and pathological lipid metabolism. In the latter case, special attention was directed to the lysosomal B-glucosidase in relation to Gaucher disease and to sphin­ gomyelinase in relation to Niemann-Pick disease, and "two round" table discussions were devoted to the two respective items. But research on many other lipidoses was presented, as lectures or posters and avidly discussed. Other topics presented in special sessions were drug induced lipidoses and peroxisomal disorders. The meeting ended with a session devoted to medical aspects of disorders of lipid metabolism. The symposium which included about 50 lectures and close to 70 posters had an intense scientific character superimposed on a most pleasant and collegial atmosphere. Ample time was provided to personal discussions ; lodging of the participants in the students' residence located near the meeting place, permitted further contacts between them.

Editors and Affiliations

  • Paul Sabatier University, Toulouse, France

    Robert Salvayre, Louis Douste-Blazy

  • Hebrew University—Hadassah School of Medicine, Jerusalem, Israel

    Shimon Gatt

Bibliographic Information

  • Book Title: Lipid Storage Disorders

  • Book Subtitle: Biological and Medical Aspects

  • Editors: Robert Salvayre, Louis Douste-Blazy, Shimon Gatt

  • Series Title: NATO Science Series A:

  • DOI: https://doi.org/10.1007/978-1-4613-1029-7

  • Publisher: Springer New York, NY

  • eBook Packages: Springer Book Archive

  • Copyright Information: Plenum Press, New York 1988

  • Softcover ISBN: 978-1-4612-8300-3Published: 27 December 2011

  • eBook ISBN: 978-1-4613-1029-7Published: 06 December 2012

  • Edition Number: 1

  • Number of Pages: 826

  • Topics: Life Sciences, general

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