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• W2805177571 abstract "Cholesteryl esters are generated at multiple sites in the body by sterol O-acyltransferase (SOAT) 1 or SOAT2 in various cell types and lecithin cholesterol acyltransferase in plasma. Esterified cholesterol and triacylglycerol contained in lipoproteins cleared from the circulation via receptor-mediated or bulk-phase endocytosis are hydrolyzed by lysosomal acid lipase within the late endosomal/lysosomal (E/L) compartment. Then, through the successive actions of Niemann-Pick C (NPC) 2 and NPC 1, unesterified cholesterol (UC) is exported from the E/L compartment to the cytosol. Mutations in either NPC1 or NPC2 lead to continuing entrapment of UC in all organs, resulting in multisystem disease, which includes hepatic dysfunction and in some cases liver failure. These studies investigated primarily whether elimination of SOAT2 in NPC1-deficient mice impacted hepatic UC sequestration, inflammation, and transaminase activities. Measurements were made in 7-wk-old mice fed a low-cholesterol chow diet or one enriched with cholesterol starting 2 wk before study. In the chow-fed mice, NPC1:SOAT2 double knockouts, compared with their littermates lacking only NPC1, had 20% less liver mass, 28% lower hepatic UC concentrations, and plasma alanine aminotransferase and aspartate aminotransferase activities that were decreased by 48% and 36%, respectively. mRNA expression levels for several markers of inflammation were all significantly lower in the NPC1 mutants lacking SOAT2. The existence of a new class of potent and selective SOAT2 inhibitors provides an opportunity for exploring if suppression of this enzyme could potentially become an adjunctive therapy for liver disease in NPC1 deficiency. NEW & NOTEWORTHY In Niemann-Pick type C1 (NPC1) disease, the entrapment of unesterified cholesterol (UC) in the endosomal/lysosomal compartment of all cells causes multiorgan disease, including neurodegeneration, pulmonary dysfunction, and liver failure. Some of this sequestered UC entered cells initially in the esterified form. When sterol O-acyltransferase 2, a cholesterol esterifying enzyme present in enterocytes and hepatocytes, is eliminated in NPC1-deficient mice, there is a reduction in their hepatomegaly, hepatic UC content, and cellular injury." @default.
• W2805177571 created "2018-06-13" @default.
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• W2805177571 date "2018-10-01" @default.
• W2805177571 modified "2023-09-25" @default.
• W2805177571 title "Niemann-Pick C1-deficient mice lacking sterol<i>O</i>-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function" @default.
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• W2805177571 cites W1505250726 @default.
• W2805177571 cites W1521847152 @default.
• W2805177571 cites W1557802196 @default.
• W2805177571 cites W1579635094 @default.
• W2805177571 cites W1599492633 @default.
• W2805177571 cites W1832765217 @default.
• W2805177571 cites W1879810571 @default.
• W2805177571 cites W1964623101 @default.
• W2805177571 cites W1965754770 @default.
• W2805177571 cites W1966095387 @default.
• W2805177571 cites W1971365671 @default.
• W2805177571 cites W1981615019 @default.
• W2805177571 cites W1989102654 @default.
• W2805177571 cites W1991639393 @default.
• W2805177571 cites W1994886732 @default.
• W2805177571 cites W1997618612 @default.
• W2805177571 cites W1998279241 @default.
• W2805177571 cites W1998420670 @default.
• W2805177571 cites W1998498590 @default.
• W2805177571 cites W2006161539 @default.
• W2805177571 cites W2013771848 @default.
• W2805177571 cites W2023565383 @default.
• W2805177571 cites W2026498402 @default.
• W2805177571 cites W2031596683 @default.
• W2805177571 cites W2034833381 @default.
• W2805177571 cites W2047753249 @default.
• W2805177571 cites W2048954674 @default.
• W2805177571 cites W2049022217 @default.
• W2805177571 cites W2058815228 @default.
• W2805177571 cites W2058877492 @default.
• W2805177571 cites W2062177817 @default.
• W2805177571 cites W2068262899 @default.
• W2805177571 cites W2072385680 @default.
• W2805177571 cites W2077428177 @default.
• W2805177571 cites W2078583436 @default.
• W2805177571 cites W2078751996 @default.
• W2805177571 cites W2080261305 @default.
• W2805177571 cites W2085578246 @default.
• W2805177571 cites W2087190566 @default.
• W2805177571 cites W2088647001 @default.
• W2805177571 cites W2092054388 @default.
• W2805177571 cites W2092282071 @default.
• W2805177571 cites W2093934810 @default.
• W2805177571 cites W2109376993 @default.
• W2805177571 cites W2109931172 @default.
• W2805177571 cites W2114570899 @default.
• W2805177571 cites W2124308944 @default.
• W2805177571 cites W2124474318 @default.
• W2805177571 cites W2136116905 @default.
• W2805177571 cites W2144952133 @default.
• W2805177571 cites W2150065552 @default.
• W2805177571 cites W2150904359 @default.
• W2805177571 cites W2151602225 @default.
• W2805177571 cites W2158964347 @default.
• W2805177571 cites W2159533867 @default.
• W2805177571 cites W2160008323 @default.
• W2805177571 cites W2163292539 @default.
• W2805177571 cites W2164903672 @default.
• W2805177571 cites W2165683666 @default.
• W2805177571 cites W2168517818 @default.
• W2805177571 cites W2169232663 @default.
• W2805177571 cites W2169792817 @default.
• W2805177571 cites W2170869887 @default.
• W2805177571 cites W2186094021 @default.
• W2805177571 cites W2289035631 @default.
• W2805177571 cites W2320064004 @default.
• W2805177571 cites W2328234883 @default.
• W2805177571 cites W2328315454 @default.
• W2805177571 cites W2339127500 @default.
• W2805177571 cites W2367049629 @default.
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• W2805177571 doi "https://doi.org/10.1152/ajpgi.00124.2018" @default.
• W2805177571 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6230690" @default.
• W2805177571 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/29878847" @default.
• W2805177571 hasPublicationYear "2018" @default.
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