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JBC, Vol. 250, Issue 21, 8487-8495, Nov, 1975
J. L. Goldstein, S. E. Dana, J. R. Faust, A. L. Beaudet and M. S. Brown
The hydrolysis of cholesteryl esters contained in plasma low density
lipoprotein was reduced in cultured fibroblasts derived from a patient with
cholesteryl ester storage disease, an inborn error of metabolism in which
lysosomal acid lipase activity is deficient. While these mutant cells
showed a normal ability to bind low density lipoprotein at its high
affinity cell surface receptor site, to take up the bound lipoprotein
through endocytosis, and to hydrolyze the protein component of the
lipoprotein in lysosomes, their defective lysosomal hydrolysis of the
cholesteryl ester component of the lipoprotein led to the accumulation
within the cell of unhydrolyzed cholesteryl esters, the fatty acid
distribution of which resembled that of plasma lipoprotein. When the
cholesteryl ester storage disease cells were incubated with low density
lipoprotein, the reduced rate of liberation of free cholesterol by these
mutant cells was associated with a delay in the occurrence of two
lipoprotein-mediated regulatory events, suppression of
3-hydroxy-3-methylglutaryl coenzyme A reductase activity, and activation of
endogenous cholesteryl ester formation. In contrast to their defective
hydrolysis of exogenously derived lipoprotein-bound cholesteryl esters, the
choleseryl ester storage disease cells showed a normal rate of hydrolysis
of cholesteryl esters that had been synthesized within the cell. These data
lend support to the concept that in cultured human fibroblasts cholesteryl
esters entering the cell bound to low density lipoprotein are hydrolyzed
within the lysosome and that one of the functions of this intracellular
organelle is to supply the cell with free cholesterol.
Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease
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