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

Pub. Date : 2014 Oct

PMID : 25115571






2 Functional Relationships(s)
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Compound Name
Protein Name
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1 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. Cholesterol NPC intracellular cholesterol transporter 1 Mus musculus
2 There was a transient increase in biliary cholesterol concentration in Npc1(-/-) mice after 2HPbetaCD. Cholesterol NPC intracellular cholesterol transporter 1 Mus musculus