Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice*

Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice*

Lysosomal acid lipase (LAL) is an essential enzyme that hydrolyzes triglycerides (TGs) and cholesteryl esters (CEs) in lysosomes. Genetic LAL mutations lead to Wolman disease (WD) and cholesteryl ester storage disease (CESD). An LAL-null (lal−/−) mouse model resembles human WD/CESD with storage of C...

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Bibliographic Details
Main Authors:	Hong Du, Terri L. Cameron, Stephen J. Garger, Gregory P. Pogue, Lee A. Hamm, Earl White, Kathleen M. Hanley, Gregory A. Grabowski
Format:	Article
Language:	English
Published:	Elsevier 2008-08-01
Series:	Journal of Lipid Research
Subjects:	cholesteryl esters triglyceride plant-produced human enzyme macrophage pharmacokinetics pharmacodynamics
Online Access:	http://www.sciencedirect.com/science/article/pii/S0022227520346824

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