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Lipases, Cholesterylesterases and Carboxylesterases in Lymphoid Cell Lines: Substrate Specificity and Relation to Wolman’s, Cholesteryl Ester Storage Diseases and Lipid Storage Myopathy

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Enzymes of Lipid Metabolism II

Part of the book series: NATO ASI Series ((NSSA,volume 116))

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Abstract

The lysosomal acid lipase hydrolyses neutral glycerides as well as cholesteryl esters1. These lipids might originate from extracellular sources (e.g. the serum lipoproteins) or be biosynthesized by the cellular enzymes. The genetic deficiency of acid lipase is the primary defect of two distinct hereditary disorders: Wolman disease and cholesteryl ester storage disease (or polycorie cholestérolique de l’adulte)2–5. Both diseases are characterized by the massive lysosomal storage of neutral lipids, but differ in clinical aspects and evolution: Wolman disease patients are affected with severe gastro-intestinal symptoms, hepatosplenomegaly, adrenal calcifications and death occurs generally in the first year of life, while cholesteryl ester storage disease is characterized by hepatosplenomegaly without adrenal invol-vement and is compatible with an adult life2,3,5.

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Author information

Authors and Affiliations

  1. Faculté de Médecine, Laboratoire de Biochimie et INSERM 101, 37 allées Jules Guesde, 31000, Toulouse, France

    R. Salvayre, A. Nègre, A. Maret, J. Radom, P. Rogalle, Q. Q. Dang & L. Douste-Blazy

  2. Dpt of Membrane Biochemistry and Neurochemistry, Hebrew University-Hadassah School of Medicine, Jerusalem, Israel

    S. Gatt

Authors
  1. R. Salvayre
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  2. A. Nègre
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  3. A. Maret
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  4. J. Radom
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  5. P. Rogalle
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  6. Q. Q. Dang
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  7. S. Gatt
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  8. L. Douste-Blazy
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Editor information

Editors and Affiliations

  1. CNRS Center for Neurochemistry and INSERM U44, Louis Pasteur University, Strasbourg, France

    Louis Freysz , Henri Dreyfus  & Raphaël Massarelli ,  & 

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

    Shimon Gatt

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© 1986 Plenum Press, New York

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Salvayre, R. et al. (1986). Lipases, Cholesterylesterases and Carboxylesterases in Lymphoid Cell Lines: Substrate Specificity and Relation to Wolman’s, Cholesteryl Ester Storage Diseases and Lipid Storage Myopathy. In: Freysz, L., Dreyfus, H., Massarelli, R., Gatt, S. (eds) Enzymes of Lipid Metabolism II. NATO ASI Series, vol 116. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-5212-9_96

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  • DOI: https://doi.org/10.1007/978-1-4684-5212-9_96

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-5214-3

  • Online ISBN: 978-1-4684-5212-9

  • eBook Packages: Springer Book Archive

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