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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Bibliografiska uppgifter
Huvudupphovsmän: Aqul, Amal, Lopez, Adam M., Posey, Kenneth S., Taylor, Anna M., Repa, Joyce J., Burns, Dennis K., Turley, Stephen D.
Materialtyp: Artigo
Språk:Inglês
Publicerad: American Physiological Society 2014
Ämnen:
Liver and Biliary Tract
Länkar: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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