Abstract
Wolman disease is a rare autosomal recessive lysosomal storage disorder characterized by a severe deficiency of the acid lipase, which induces a massive neutral lipid storage (triacylglycerols and cholesteryl esters) (1, 2). A lymphoid cell line, established by Epstein-Barr virus transformation of blood B lymphocytes from a patient affected with Wolman disease, expressed the characteristic features of the disease, (lysosomal acid lipase deficiency and accumulation of neutral lipids) (3, 4). We have recently demonstrated that the neutral lipids accumulated in the lysosomes of Wolman fibroblasts are mainly from exogenous (lipoproteins) origin, whereas endogenously biosynthesized neutral lipids do not participate at appreciable level to the lysosomal storage (5).
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© 1988 Plenum Press, New York
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Nègre, A., Livni, N., Maret, A., Douste-Blazy, L., Salvayre, R. (1988). Metabolism of Extracellular Triacylglycerols (From Lipoproteins) in a Woman Lymphoid Cell Line. 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_38
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DOI: https://doi.org/10.1007/978-1-4613-1029-7_38
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