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Current Trends in Sphingolipidoses and Allied Disorders

  • Book
  • © 1976

Overview

Editors:
  1. Bruno W. Volk

    1. Kingsbrook Jewish Medical Center, Isaac Albert Research Institute, USA
      Downstate Medical Center, State University of New York, USA

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  2. Larry Schneck

    1. Downstate Medical Center, State University of New York, USA
      Neuroscience Center, Kingsbrook Jewish Medical Center, USA
      Birth Defects Center, Isaac Albert Research Institute, USA

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Part of the book series: Advances in Experimental Medicine and Biology (AEMB, volume 68)

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Table of contents (38 chapters)

  1. Front Matter

    Pages i-xi
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  2. Simple Ultra-Microtechniques for Genetic Complementation Analysis and Early Prenatal Diagnosis of Sphingolipidoses

    • P. Hösli
    Pages 1-13

  3. Storage and Excretion of Oligosaccharides and Glycopeptides in the Gangliosidoses

    • Leonhard S. Wolfe, N. M. K. Ng Ying Kin
    Pages 15-29

  4. Glycoprotein Catabolism in Brain Tissue in the Lysosomal Enzyme Deficiency Diseases

    • Eric G. Brunngraber, Leonard G. Davis, Javaid I. Javaid, Bruno Berra
    Pages 31-48

  5. Component forms of Acid Hydrolases in Subcellular Granules from Human Leucocytes

    • R. B. Ellis, A. D. Patrick
    Pages 49-62

  6. Fetal Pathology and Ultrastructure of Neuropathic Gaucher’s Disease

    • Shigehiko Kamoshita, Mariko Odawara, Mariko Yoshida, Misao Owada, Teruo Kitagawa
    Pages 63-75

  7. Recent Observations on Gaucher’s Disease

    • J. N. Kanfer, S. S. Raghaven, R. A. Mumford, J. Sullivan, C. Spielvogel, G. Legler et al.
    Pages 77-98

  8. Studies on the Pathogenesis of Krabbe’s Leukodystrophy: Cellular Reaction of the Brain to Exogenous Galactosylsphingosine, Monogalactosyl Diglyceride, and Lactosylceramide

    • Kinuko Suzuki, Harumi Tanaka, Kunihiko Suzuki
    Pages 99-114

  9. Chemical Pathology of Krabbe Disease: The Occurrence of Psychosine and Other Neutral Sphingoglycolipids

    • Marie-Thérèse Vanier, Lars Svennerholm
    Pages 115-126

  10. Glycolipid Metabolism in the Canine form of Globoid Cell Leukodystrophy

    • Elvira Costantino-Ceccarini, Thomas F. Fletcher, Kunihiko Suzuki
    Pages 127-145

  11. Fucosidosis: Clinical, Pathologic, and Biochemical Studies of Five Patients

    • Benjamin H. Landing, George N. Donnell, Omar S. Alfi, Harry B. Neustein, Fred A. Lee, Won G. Ng et al.
    Pages 147-165

  12. Variability of Expressivity of α-Fucosidase Deficiency

    • Vimalkumar Patel, Wolfgang Zeman
    Pages 167-186

  13. Fucosidosis

    • Glyn Dawson, Grace Chen Tsay
    Pages 187-203

  14. Alpha-L-Fucosidase in Normal and Deficient Individuals

    • Kurt Hirschhorn, Nicholas G. Beratis, Bryan M. Turner
    Pages 205-223

  15. Studies in Metachromatic Leukodystrophy. XIII. Purification of Sulfatase A from Normal Human Liver

    • Janet Collins, Warren Yamada, William Worth, James Austin
    Pages 225-232

  16. p.Nitrocatechol Sulfate for Arylsulfatase Assay: Detection of Metachromatic Leukodystrophy Variants

    • Gisèle Dubois, Jean-Claude Turpin, Nicole Baumann
    Pages 233-237

  17. Arylsulfatases A and B in Metachromatic Leukodystrophy and Maroteaux-Lamy Syndrome: Studies with 4-Methylumbelliferyl Sulfate

    • Edwin H. Kolodny, Richard A. Mumford
    Pages 239-251

  18. Iduronate Sulfatase Determination for the Diagnosis of the Hunter Syndrome and the Detection of the Carrier State

    • Elizabeth F. Neufeld, Ingeborg Liebaers, Timple W. Lim
    Pages 253-260

  19. The Enzymic Defects in Morquio and Maroteaux-Lamy Syndrome

    • Albert Dorfman, Bradley Arbogast, Reuben Matalon
    Pages 261-276

  20. Mannosidosis: Studies of the α-D-Mannosidase Isozymes in Health and Disease

    • R. J. Desnick, L. L. Walling, P. M. Anderson, M. K. Raman, H. L. Sharp, J. U. Ikonne
    Pages 277-299
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Keywords

About this book

The present volume contains the scientific contributions to the Fifth International Symposium on "Current Trends in Sphingo­ lipidoses and Allied Disorders" under the auspices of the Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center, the Department of Pathology, Downstate Medical Center, State Uni­ versity of New York, Brooklyn, New York, and the National Tay-Sachs and Allied Diseases Association, Inc., New York. A review of the four previous Symposia shows the increase in scope of the scientific exploration in this rapidly expanding field. The first meeting, held in 1958, was devoted to the discussion al­ most entirely of Tay-Sachs disease. The majority of the work emanated from local laboratories. The participants at the present Symposium came from many other domestic and foreign research in­ stitutions. The scope of the papers presented at these meetings and the interest shown in the Symposium demonstrates the signifi­ cance attached by the scientific community to the problems of these hereditary diseases. The reasons for this are apparent, when one considers the contributions during recent years to our basic know­ ledge by lipid and enzyme chemistry, genetics, and neuropathology. Partly because of the hereditary nature of these diseases any new discovery in this field has general meaning and permits cautious generalization well beyond its clinical significance.

Editors and Affiliations

  • Kingsbrook Jewish Medical Center, Isaac Albert Research Institute, USA

    Bruno W. Volk

  • Downstate Medical Center, State University of New York, USA

    Bruno W. Volk, Larry Schneck

  • Neuroscience Center, Kingsbrook Jewish Medical Center, USA

    Larry Schneck

  • Birth Defects Center, Isaac Albert Research Institute, USA

    Larry Schneck

Bibliographic Information

  • Book Title: Current Trends in Sphingolipidoses and Allied Disorders

  • Editors: Bruno W. Volk, Larry Schneck

  • Series Title: Advances in Experimental Medicine and Biology

  • DOI: https://doi.org/10.1007/978-1-4684-7735-1

  • 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 1976

  • Softcover ISBN: 978-1-4684-7737-5Published: 26 November 2012

  • eBook ISBN: 978-1-4684-7735-1Published: 09 March 2013

  • Series ISSN: 0065-2598

  • Series E-ISSN: 2214-8019

  • Edition Number: 1

  • Number of Pages: XII, 612

  • Number of Illustrations: 70 b/w illustrations

  • Topics: Pathology

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