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Sphingolipids, Sphingolipidoses and Allied Disorders

Proceedings of the Symposium on Sphingolipidoses and Allied Disorders held in Brooklyn, New York, October 25–27, 1971

  • Conference proceedings
  • © 1972

Overview

Editors:
  1. Bruno W. Volk

    1. Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center and Department of Pathology, Downstate Medical Center, State University of New York, Brooklyn, USA

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  2. Stanley M. Aronson

    1. Department of Pathology, Miriam Hospital and Brown University, Providence, USA

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Part of the book series: Advances in Experimental Medicine and Biology (AEMB, volume 19)

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Table of contents (48 papers)

  1. Front Matter

    Pages i-xxvii
    Download chapter PDF

  2. Fine Structure of Early Tay-Sachs Disease

    • M. Adachi, J. Torii, L. Schneck, B. W. Volk
    Pages 1-13

  3. Biopsy Diagnosis of Lipidoses: Background Considerations, General Concepts and Practical Aspects

    • Benjamin H. Landing, Harry B. Neustein, Shigehiko Kamoshita
    Pages 15-35

  4. Lysosomal Diseases and Fibroblast Cultures: Biochemical and Electron Microscopic Observations

    • George Hug, William K. Schubert, Shirley Soukup
    Pages 37-51

  5. The Morphogenesis and Biochemical Characteristics of Ceroid Isolated from Cases of Neuronal Ceroid-Lipofuscinosis

    • Aristotle N. Siakotos, Hans H. Goebel, Vimalkumar Patel, Itaru Watanabe, Wolfgang Zeman
    Pages 53-61

  6. Effect of Conditions of Extraction on the Extractability of Brain Gangliosides

    • J. Folch-Pi
    Pages 63-76

  7. Gangliosides of CSF and Plasma: Their Relation to the Nervous System

    • R. W. Ledeen, R. K. Yu
    Pages 77-93

  8. Ganglioside Inner Esters

    • Robert H. McCluer, James E. Evans
    Pages 95-102

  9. Human Brain Lipid Composition Changes with Age and Alterations in Some Pathological States: A New Method of Graphic Analysis

    • George Rouser, Gene Kritchevsky
    Pages 103-126

  10. Brain Glycoproteins and Inter-Cell Recognition: Tay-Sachs’ Disease and Intraneuronal Recognition

    • Samuel Bogoch
    Pages 127-149

  11. A Fast Moving Protein in Tay-Sachs Disease

    • Denise Karcher, A. Lowenthal, W. Zeman
    Pages 151-162

  12. Effect of Amino Acid Imbalance on Polyribosome Profiles and Protein Synthesis in Fetal Cerebral Cortex

    • Paul W. K. Wong, Parvin Justice
    Pages 163-174

  13. Purification and Properties of Two Sphingolipid Hydrolases

    • Howard R. Sloan, Jan L. Breslow, Donald S. Fredrickson
    Pages 175-186

  14. Deficiency of Specific Proteins in the Inborn Errors of Mucopolysaccharide Metabolism

    • Elizabeth F. Neufeld, Robert W. Barton, Michael Cantz, Jeffery G. Derge, Clara W. Hall, Hans Kresse et al.
    Pages 187-194

  15. The Degradation of Acid Mucopolysaccharides and the Mucopolysaccharidoses

    • Albert Dorfman, Reuben Matalon, J. Anthony Cifonelli, Jerry Thompson, Glyn Dawson
    Pages 195-210

  16. The Mucopolysaccharidoses as Lysosomal Diseases

    • F Van Hoof, H. G. Hers
    Pages 211-223

  17. Recent Observations on Gaucher’s Disease

    • Julian N. Kanfer, Marcia Stein, Christine Spielvogel
    Pages 225-236

  18. Interaction of Enzymes with Lipid Substrates

    • Shimon Gatt, Yechezkel Barenholz, Ilana Borkovski-Kubiler, Zelina Leibovitz-Ben Gershon
    Pages 237-256

  19. Problems in Prenatal Diagnosis Using Sphingolipid Hydrolase Assays

    • J. Alexander Lowden, Marie-Anne LaRamee
    Pages 257-267

  20. Radioactive Precursor Incorporation into Lipids of Humans with Cerebral Lipidoses:1-14C-Glucosamine, U3H-Serine, and 3H-Acetate

    • Robert M. Burton, S. Handa, R. E. Howard, T. Vietti
    Pages 269-276
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Keywords

About this book

This text contains the scientific contributions to the Fourth International Symposium on Sphingolipids, Sphingo­ lipidoses and Allied Disorders held at the Kingsbrook Jewish Medical Center on October 25-27, 1971. These meetings were conducted under the auspices of the Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center and the National Tay-Sachs and Allied Diseases Association, Inc. Four symposia, held in 1958, 1961, 1965 and 1971 were designed to gather the most relevant and innovative of the laboratory and field studies concerned with these hereditary disorders. The texts generated by these periodic meetings have mirrored the increasing absorption of the scientific community in the problems of sphingolipid metabolism. The first meeting in 1958 consisted of but twelve pre­ sentations, the majority emanating from local laboratories. The current sessions contain 48 scientific presentations by scientists from nine countries and demonstrate the increas­ ingly diversified techniques and approaches employed in the study of these diseases. Many of the authors, in exploring data on the mucopolysaccharidoses and leucodystrophies, as well as the sphingolipidoses, have given recognition to those biochemical areas held in common by these otherwise diverse disease processes. The problems of prevention and therapy of these diseases have been considered by some of the contributors. Laboratory screening procedures designed to detect carriers of the va­ rious lipidoses are now available and the experiences of some laboratories in this area are summarized within this volume. The prospective identification of heterozygotes may indeed become a powerful adjunct in genetic counseling.

Editors and Affiliations

  • Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center and Department of Pathology, Downstate Medical Center, State University of New York, Brooklyn, USA

    Bruno W. Volk

  • Department of Pathology, Miriam Hospital and Brown University, Providence, USA

    Stanley M. Aronson

Bibliographic Information

  • Book Title: Sphingolipids, Sphingolipidoses and Allied Disorders

  • Book Subtitle: Proceedings of the Symposium on Sphingolipidoses and Allied Disorders held in Brooklyn, New York, October 25–27, 1971

  • Editors: Bruno W. Volk, Stanley M. Aronson

  • Series Title: Advances in Experimental Medicine and Biology

  • DOI: https://doi.org/10.1007/978-1-4757-6570-0

  • 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 1972

  • Softcover ISBN: 978-1-4757-6572-4Published: 03 March 2013

  • eBook ISBN: 978-1-4757-6570-0Published: 17 April 2013

  • Series ISSN: 0065-2598

  • Series E-ISSN: 2214-8019

  • Edition Number: 1

  • Number of Pages: XXVII, 691

  • Number of Illustrations: 154 b/w illustrations

  • Topics: Cardiology

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