Deficient Activity of Hepatic Acid Lipase in Cholesterol Ester Storage Disease

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Science  21 Apr 1972:
Vol. 176, Issue 4032, pp. 309-310
DOI: 10.1126/science.176.4032.309


Absence of lysosomal acid lipase activity in the liver is described in cholesterol ester storage disease and Wolman's disease. This enzyme deficiency may result in the excess hepatic cholesterol ester found in both conditions. However, clinical, genetic, and histopathologic differences suggest that the two conditions are separate diseases not completely explained by deficient enzyme activity.