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. 1972 Apr 21;176(4032):309-10.
doi: 10.1126/science.176.4032.309.

Deficient activity of hepatic acid lipase in cholesterol ester storage disease

J A Burke, W K Schubert

Deficient activity of hepatic acid lipase in cholesterol ester storage disease

J A Burke et al. Science. .

Abstract

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.

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