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Cholesteryl ester storage disease and Wolman disease: phenotypic variants of lysosomal acid cholesteryl ester hydrolase deficiency.

The lysosomal enzyme responsible for cholesteryl ester hydrolysis, acid cholesteryl ester hydrolase, or acid lipase (E.C.3.1.1.13) plays an important role in cellular cholesterol metabolism. Loss of the activity of this enzyme in tissues of individuals with both Wolman disease and cholesteryl ester...

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Dettagli Bibliografici
Autori principali: Hoeg, J M, Demosky, S J, Pescovitz, O H, Brewer, H B
Natura: Artigo
Lingua:Inglês
Pubblicazione: 1984
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC1684644/
https://ncbi.nlm.nih.gov/pubmed/6097111
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