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Science 21 April 1972:
Vol. 176. no. 4032, pp. 309 - 310
DOI: 10.1126/science.176.4032.309
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Articles

Deficient Activity of Hepatic Acid Lipase in Cholesterol Ester Storage Disease

Joseph A. Burke 1 and William K. Schubert 1

1 Department of Pediatrics, Children's Hospital Research Foundation, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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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