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Sphingolipids, Sphingolipidoses and Allied Disorders pp 429–450Cite as

Liver Glycolipids, Steroid Sulfates and Steroid Sulfatases in a Form of Metachromatic Leukodystrophy Associated with Multiple Sulfatase Deficiencies

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

Abstract

Austin et al. (4) and Mehl and Jatzkewitz et al. (14,22) have shown that metachromatic leukodystrophy (MLD) is associated with deficient activity of arylsulfatase A. This enzyme, in combination with a heat-stable complementary factor, cleaves sulfate from sulfatide.(22,23) Arylsulfatase A deficiency thus causes the sulfatide accumulation which is pathognomic of this disease.

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Authors and Affiliations

  1. The Eunice Kennedy Shriver Center at the Walter E. Fernald State School, Waverley, Massachusetts, 02178, USA

    H. W. Moser, M. Sugita, M. D. Harbison & M. Williams

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  1. H. W. Moser
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  2. M. Sugita
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  3. M. D. Harbison
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  4. M. Williams
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Editor information

Editors and Affiliations

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

    Bruno W. Volk

  2. Department of Pathology, Miriam Hospital and Brown University, Providence, Rhode Island, USA

    Stanley M. Aronson

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Moser, H.W., Sugita, M., Harbison, M.D., Williams, M. (1972). Liver Glycolipids, Steroid Sulfates and Steroid Sulfatases in a Form of Metachromatic Leukodystrophy Associated with Multiple Sulfatase Deficiencies. In: Volk, B.W., Aronson, S.M. (eds) Sphingolipids, Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 19. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6570-0_30

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  • DOI: https://doi.org/10.1007/978-1-4757-6570-0_30

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4757-6572-4

  • Online ISBN: 978-1-4757-6570-0

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