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Sialidase Deficiency in the SM/J Mouse: A Physically Altered Mutant Enzyme in the Liver

  • Conference paper
Lipid Storage Disorders

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

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Abstract

The glycosidase sialidase (acylneuraminyl glycohydrolase, EC 3.2.1.18) hydrolyzes terminal sialic acid residues of glycoproteins, oligosaccharides and glycolipids, and is widely distributed in viruses, bacteria and mammals.1 Recessively inherited syndromes characterized by sialidase deficiency, the sialidoses, have been described in man2–4 and genetic sialidase deficiency has been reported in the mouse strain SM/J5,6 and the rat strain KGH.7 In SM/J mice, the deficiency involves a labile component (named A) which denatures rapidly at 0°C.5

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Author information

Authors and Affiliations

  1. Section de Génétique Médicale, Hôpital Sainte-Justine, Université de Montréal, Montréal, Canada, H3T 1C5

    Denise Lu Shun Yan, Guy Beauregard & Michel Potier

  2. Department of Veterinary Pathology, Texas A and M University, College Station, Texas, 77843, USA

    James E. Womack

Authors
  1. Denise Lu Shun Yan
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  2. James E. Womack
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  3. Guy Beauregard
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  4. Michel Potier
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Editor information

Editors and Affiliations

  1. Paul Sabatier University, Toulouse, France

    Robert Salvayre  & Louis Douste-Blazy  & 

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

    Shimon Gatt

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© 1988 Plenum Press, New York

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Lu Shun Yan, D., Womack, J.E., Beauregard, G., Potier, M. (1988). Sialidase Deficiency in the SM/J Mouse: A Physically Altered Mutant Enzyme in the Liver. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_82

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  • DOI: https://doi.org/10.1007/978-1-4613-1029-7_82

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4612-8300-3

  • Online ISBN: 978-1-4613-1029-7

  • eBook Packages: Springer Book Archive

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