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...

Full description

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

Internet

http://www.sciencedirect.com/science/article/pii/S0022227520346824

Similar Items