Loading...

Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function

In Niemann-Pick type C (NPC) disease, loss of function mutations in either NPC1 or NPC2 result in progressive accumulation of unesterified cholesterol (UC) and glycosphingolipids in all organs leading to neurodegeneration, pulmonary dysfunction and sometimes liver failure. There is no cure for this...

Fuld beskrivelse

Na minha lista:
Bibliografiske detaljer
Udgivet i:Clin Exp Pharmacol Physiol
Main Authors: Lopez, Adam M., Terpack, Sandi Jo, Posey, Kenneth S., Liu, Benny, Ramirez, Charina M., Turley, Stephen D.
Format: Artigo
Sprog:Inglês
Udgivet: 2014
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4526211/
https://ncbi.nlm.nih.gov/pubmed/25115571
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/1440-1681.12285
Tags: Tilføj Tag
Ingen Tags, Vær først til at tagge denne postø!