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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...

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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:Clin Exp Pharmacol Physiol
Prif Awduron: Lopez, Adam M., Terpack, Sandi Jo, Posey, Kenneth S., Liu, Benny, Ramirez, Charina M., Turley, Stephen D.
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: 2014
Pynciau:
Mynediad Ar-lein: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
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