Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice

Cholesteryl ester storage disease (CESD) results from loss-of-function mutations in LIPA, the gene that encodes lysosomal acid lipase (LAL). Hepatomegaly and deposition of esterified cholesterol (EC) in multiple organs ensue. The present studies quantitated rates of synthesis, absorption, and dispos...

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Detalhes bibliográficos
Main Authors: Aqul, Amal, Lopez, Adam M., Posey, Kenneth S., Taylor, Anna M., Repa, Joyce J., Burns, Dennis K., Turley, Stephen D.
Formato: Artigo
Idioma:Inglês
Publicado em: American Physiological Society 2014
Assuntos:
Liver and Biliary Tract
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4200320/
https://ncbi.nlm.nih.gov/pubmed/25147230
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajpgi.00243.2014
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