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...

ver descrição completa

Na minha lista:
Detalhes bibliográficos
Main Authors: Du, Hong, Cameron, Terri L., Garger, Stephen J., Pogue, Gregory P., Hamm, Lee A., White, Earl, Hanley, Kathleen M., Grabowski, Gregory A.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2008
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2444013/
https://ncbi.nlm.nih.gov/pubmed/18413899
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.M700482-JLR200
Tags: Adicionar Tag
Sem tags, seja o primeiro a adicionar uma tag!

Registos relacionados