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

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

Abstract

Metachromatic leukodystrophy (MLD), a sphingolipidosis, and Maroteaux-Lamy syndrome (MLS), a mucopolysaccharidosis, are genetically distinct diseases which have in common a markedly impaired ability to cleave the artificial substrate p-nitrocatechol sulfate (NCS) under certain specified conditions. Numerous studies employing this substrate have demonstrated deficiency of arylsulfatase A (AS-A) in MLD (1,6,7) and of arylsulfatase B (AS-B) in MLS(3, 4, 7, 13).However, several problems exist in connection with its use.

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References

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© 1976 Plenum Press, New York

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Kolodny, E.H., Mumford, R.A. (1976). Arylsulfatases A and B in Metachromatic Leukodystrophy and Maroteaux-Lamy Syndrome: Studies with 4-Methylumbelliferyl Sulfate. In: Volk, B.W., Schneck, L. (eds) Current Trends in Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 68. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7735-1_16

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  • DOI: https://doi.org/10.1007/978-1-4684-7735-1_16

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-7737-5

  • Online ISBN: 978-1-4684-7735-1

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