Abstract
Wolman disease is an inherited lysosomal storage disorder characterized by a severe deficiency in acid lipase and a lysosomal storage of neutral lipids (1, 2). Acid lipase deficiency can be demonstrated by using natural or synthetic analogues (3, 4), but it is well known that synthetic methylumbelliferyl acyl esters (which are widely utilized for the determination of acid lipase activity) are also hydrolyzed by non specific carboxylesterases (5).
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References
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© 1988 Plenum Press, New York
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Dang, Q.Q. et al. (1988). Microscale Synthesis Of Fluorescent Cholesteryl Esters for the Study of Lysosomal Cholesteryl Esterases and Diagnosis of Wolman Disease. 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_61
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DOI: https://doi.org/10.1007/978-1-4613-1029-7_61
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