Niemann-Pick C1 Modulates Hepatic Triglyceride Metabolism and Its Genetic Variation Contributes to Serum Triglyceride Levels

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Niemann-Pick C1 modulates hepatic triglyceride metabolism and its genetic variation contributes to serum triglyceride levels.

OBJECTIVE To study how Niemann-Pick disease type C1 (NPC1) influences hepatic triacylglycerol (TG) metabolism and to determine whether this is reflected in circulating lipid levels. METHODS AND RESULTS In Npc1(-/-) mice, the hepatic cholesterol content is increased but the TG content is decreased. We investigated lipid metabolism in Npc1(-/-) mouse hepatocytes and the association of NPC1 sing...

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Niemann-Pick Disease Type C1 Modulates Hepatic Triglyceride Metabolism and Its Genetic Variation Contributes to Serum Triglyceride Levels

to determine whether this is reflected in circulating lipid levels. Methods and Results—In Npc1 / mice, the hepatic cholesterol content is increased but the TG content is decreased. We investigated lipid metabolism in Npc1 / mouse hepatocytes and the association of NPC1 single-nucleotide polymorphisms with circulating TGs in humans. TGs were reduced in Npc1 / mouse serum and hepatocytes. In Npc...

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Niemann-Pick C1

So what does NPC1 really do? This is the key question. NPC1 shares significant structural homology with the resistance–nodulation–division (RND) family of permeases, members of which transport a number of substrates including heavy metals and lipids. Like its prokaryotic counterparts, NPC1 appears to transport fatty acids, but has not yet been shown to transport cholesterol. It has been suggest...

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Hepatic ABC transporters and triglyceride metabolism.

PURPOSE OF REVIEW Elevated plasma triglyceride and reduced HDL concentrations are prominent features of metabolic syndrome and type 2 diabetes. Individuals with Tangier disease also have elevated plasma triglyceride concentrations and very low HDL, resulting from mutations in ATP-binding cassette transporter A1 (ABCA1), an integral membrane protein that facilitates nascent HDL particle assembly...

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Triglyceride-rich lipoprotein remnant levels and metabolism

mong the major components of the standard plasma lipid rofile, the total triglyceride (TG) level is generally considred the weakest risk factor for atherosclerosis, for which here are several likely contributing reasons. First, TG is eadily metabolized and is not a major constituent of therosclerotic plaque. Second, plasma TG varies greatly rom day to day in the fasting state and even more in t...

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ژورنال

عنوان ژورنال: Arteriosclerosis, Thrombosis, and Vascular Biology

سال: 2010

ISSN: 1079-5642,1524-4636

DOI: 10.1161/atvbaha.110.207191